Background:anti-NXP2 antibodies is considered a serological marker of dermatomyositis (DM), with calcinosis, severe myositis and, in some series, cancer. Historically, these associations have been detected with immunoprecipitation (IP),... more
Background:anti-NXP2 antibodies is considered a serological marker of dermatomyositis (DM), with calcinosis, severe myositis and, in some series, cancer. Historically, these associations have been detected with immunoprecipitation (IP), but in the last few years commercial lineblot (LB) assay have been released.Objectives:to analyze the clinical features associated to anti-NXP2 antibodies, including the onset of concomitant cancers, both with LB and homemade IPMethods:clinical and serological data from medical charts of 213 patients with a diagnosis of inflammatory miosidites without anti-NXP2 (NXP2-), followed-up by two third-level Centers, and 61 anti-NXP2+ patients from 10 Rheumatological centers were analyzed. Anti-myositis specific (MSA) and anti-myositis associated antibodies (MAA) were detected in single centers by LB (Euroimmun Autoimmune Inflammatory Myopathies 16 antigens). Anti-NXP2 was confirmed by protein and RNA IP, as previously described (1)Results:clinical diagnosis...
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Objectives: Women with Rheumatoid Arthritis (RA) can experience flares during pregnancy that might influence pregnancy outcomes. We aimed at assessing the disease course during pregnancy and identifying risk factors for flares.Methods:... more
Objectives: Women with Rheumatoid Arthritis (RA) can experience flares during pregnancy that might influence pregnancy outcomes. We aimed at assessing the disease course during pregnancy and identifying risk factors for flares.Methods: Data about prospectively-followed pregnancies in RA were retrospectively collected before conception, during each trimester and in the post-partum period. Clinical characteristics, disease activity (DAS28-CRP3), medication use, and pregnancy outcomes were analysed with regard to disease flares.Results: Among 73 women who had a live birth, 64 (88%) were in remission/low disease activity before conception. During pregnancy, a flare occurred in 27 (37%) patients, mainly during first and second trimester. Flares during pregnancy were associated with the discontinuation of bDMARDs at positive pregnancy test (55% of patients with flare vs. 30% of patients with no flare, p 0.034, OR 2.857, 95% CI 1.112–8.323) and a previous use of >1 bDMARDs (33% of patie...
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BACKGROUND despite the absence of specific guidelines, the treatment with intravenous immunoglobulins (IvIg) is considered effective in patients with refractory idiopathic inflammatory myopathies (IIM). The aim of our study is to evaluate... more
BACKGROUND despite the absence of specific guidelines, the treatment with intravenous immunoglobulins (IvIg) is considered effective in patients with refractory idiopathic inflammatory myopathies (IIM). The aim of our study is to evaluate the effectiveness and the safety of IvIg and define the possible profile of IIM patients candidate to IvIg treatment. METHODS we performed a retrospective study of IIM pts. treated with IvIg (2 g/kg/month). We collected demographic, epidemiological, laboratory and clinical data. Additionally, to evaluate the toxicity, the adverse events occurred during the treatment were collected. RESULTS 123 patients with IIM were included in the study. The main indications for the prescription of IvIg were muscle (83.7% of patients) and esophageal involvement (45.5% of patients). IvIg were started mainly for refractory disease. At the end of treatment (mean duration 14 months), muscular necrosis enzymes decreased significantly and dysphagia VAS decreased significantly (p < 0.001), while MMT value increased (104.6 ± 24.2 vs. 127.0 ± 22.2 p < 0.001). Ninety-six pts. (78%) responded to IvIg. They had a shorter disease duration (p < 0.001), higher creatine kinase levels (p < 0.001), and higher prevalence of myalgias at the baseline (p = 0.023) compared to non-responders. The presence of Raynaud's phenomenon (p = 0.023-odds ratio 0.28 [0.11-0.72]) and skin involvement (p = 0.004, odds ratio 0.18 [0.06-0.55]), were associated to a worse response. Adverse events were mostly mild and transitory. CONCLUSIONS Despite their high cost, IvIg confirmed their effectiveness in refractory IIM pts., particularly in muscular and esophageal manifestations. Specific clinical characteristics at the baseline may identify the patients with higher probability of response to the treatment.
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Autoantibodies are powerful diagnostic tools in idiopathic inflammatory myopathies, especially for con-firming the diagnosis and contributing to the definition of disease subsets. They are present in over 80 % of patients with... more
Autoantibodies are powerful diagnostic tools in idiopathic inflammatory myopathies, especially for con-firming the diagnosis and contributing to the definition of disease subsets. They are present in over 80 % of patients with immuno-mediated myositis and directed towards ubiquitously expressed intracellular complexes. Most of these autoantibodies are reported also in other autoimmune diseases, while some are considered myositis-specific. Myositis autoantibodies are traditionally categorized in two groups, based on their diagnostic accuracy: myositis-spe-cific antibodies (MSA) and myositis-associated antibodies (MAA), the latter mostly occurring in myositis-overlap syndromes. Besides the so-called traditional MSA, including anti-synthetases, anti-SRP and anti-Mi-2 anti-bodies, additional newly conceived immune targets have been recently identified, mostly in patients with severe forms of dermatomyositis or necrotizing myopathy. They mainly encompass enzymatic proteins essentially in...
Background: Pregnant patients (pts) with spondyloarthritis (SpA) seem at increased risk for adverse pregnancy outcomes (APO), however limited and conflicting data have been published so far and risk factors for APO in these pts remain... more
Background: Pregnant patients (pts) with spondyloarthritis (SpA) seem at increased risk for adverse pregnancy outcomes (APO), however limited and conflicting data have been published so far and risk factors for APO in these pts remain poorly understood. Objectives: To assess APO and identify possible risk factors for those in a cohort of SpA pregnant pts. Methods: Data on SpA pts prospectively-followed in a pregnancy clinic from 2010 to 2019 were retrospectively analysed before conception and during each trimester. Pregnancies complicated by APO were compared with those that were uneventful for demographic and clinical variables. Active disease was defined as a DAS-28-CRP>3.2 or an ASDAS-CRP ≥ 2.1 according to peripheral or axial dominant disease respectively. Results: 56 pregnancies (mean age 34±5 years; median disease duration 60 months, IQR 24-123) in 47 pts were analysed: 37 psoriatic arthritis, 7 axial SpA, 6 undifferentiated SpA, 3 enteropathic SpA, 2 reactive arthritis and...
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IntroductionCovid-19 infection poses a serious challenge for immune-compromised patients with inflammatory autoimmune systemic diseases. We investigated the clinical-epidemiological findings of 1641 autoimmune systemic disease Italian... more
IntroductionCovid-19 infection poses a serious challenge for immune-compromised patients with inflammatory autoimmune systemic diseases. We investigated the clinical-epidemiological findings of 1641 autoimmune systemic disease Italian patients during the Covid-19 pandemic.MethodThis observational multicenter study included 1641 unselected patients with autoimmune systemic diseases from three Italian geographical areas with different prevalence of Covid-19 [high in north (Emilia Romagna), medium in central (Tuscany), and low in south (Calabria)] by means of telephone 6-week survey. Covid-19 was classified as (1)definitediagnosis of Covid-19 disease: presence of symptomatic Covid-19 infection, confirmed by positive oral/nasopharyngeal swabs; (2)highly suspectedCovid-19 disease: presence of highly suggestive symptoms, in absence of a swab test.ResultsA significantly higher prevalence of patients withdefinitediagnosis of Covid-19 disease,or withhighly suspectedCovid-19 disease, or both ...
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ABSTRACTBackground and objectiveSystemic small vessel vasculitides carry an increased risk of acute arterial and venous thromboembolic events (AVTE); however, this risk has not been systematically explored in Eosinophilic Granulomatosis... more
ABSTRACTBackground and objectiveSystemic small vessel vasculitides carry an increased risk of acute arterial and venous thromboembolic events (AVTE); however, this risk has not been systematically explored in Eosinophilic Granulomatosis with Polyangiitis (EGPA). This study assessed the occurrence and main risk factors of AVTE among EGPA patients as compared to the general community from the population-based Bruneck cohort.MethodsWe conducted a retrospective multicenter cohort study on 573 EGPA patients. Clinical and serological data were collected at diagnosis. Occurrence of AVTE and time to the first AVTE after EGPA diagnosis were recorded. Age-standardized event rate (SER) of AVTE as compared to the reference cohort was assessed. Cox regression was applied to identify AVTE predictors.Results129 EGPA patients (22.5%) had AVTE, considered as potentially life-threatening in 55.8%. Seventy patients experienced an AVTE prior to diagnosis (of whom 58.6% in the two years before diagnosis...
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ABSTRACT Background Anti-Ku antibodies (anti-Ku) are frequently detected in patients with Systemic Sclerosis (SSc),Myositis or Overlap Syndromes. Raynaud’s phenomenon (RP) is one of the main features associated with these antibodies.... more
ABSTRACT Background Anti-Ku antibodies (anti-Ku) are frequently detected in patients with Systemic Sclerosis (SSc),Myositis or Overlap Syndromes. Raynaud’s phenomenon (RP) is one of the main features associated with these antibodies. Objectives To evaluate the involvement of the microcirculation in patients with anti-Ku. Methods Anti extractable nuclear antigen (ENA) antibodies including anti-Ku were searched by counterimmunoelectrophoresis in our laboratory. Among the sera analyzed from 1995 to 2011, 51 resulted positive for anti-Ku.Clinical data were retrospectively obtained from clinical charts.The evaluation of microcirculation was made by NC through a stereoscopic microscope (Wild Heerbrugg M8, Leitz, Germany) and videocapillaroscopy (DS Medigroup, Italy). The patterns of capillary changes were defined according to Cutolo et al. [1] Results Of the 51 patients with anti-Ku,clinical charts were available for 40 patients,that were included in this study.Overlap Syndrome were diagnosed in 15 patients: 11 had Polimyositis (PM) or Dermatomyositis in overlap with SSc (10 cases) or Systemic Lupus Erythematosus (SLE) (1 case). 11 patients had an undifferentiated connective tissue disease,4 a SLE,4 a Sjogren Syndrome, 3 a PM and 1 each had a SSc, a Rheumatoid Arthritis and an Antiphospholipid Syndrome.Anti-Ku were in association with other antinuclera specificities in 11 out of 40 sera (27%), while in 29 patients (73%) were isolated. Associated antibodies were: anti-SSA/Ro in 10 sera,anti-SSB/La in 2 and anti-Ki in 3.RP was present in 32 (80%) out of 40 patients.28 patients were studied by NC.A scleroderma pattern was present in 9 patients (32%), non specific alterations in 11 patients(39%), while 8 patients (28%) had a normal NC. Sclerodermapattern was statistically associated (p=0.001, Fisher’s Exact test) with scleroderma spectrum disease (SSc or overlap including SSc). Table 1 shows the details of NC. Only 1 patients without RP referred other vascular symptoms (acrocianosis). Only 1 patient (overlap PM-SSc syndrome) out of the 40 studied experienced digital ulcers. We did not find either any association between isolated or associated anti-Ku and RP, scleroderma pattern or NC alterations. Conclusions RP is a common feature in patients with anti-Ku and most patients present NC alterations.Non specific alterations are more common,but scleroderma pattern is present in a considerable number of patients,in particular those affected by SSc spectrum diseases, even if more rare NC alterations can be present also in patients without RP.Despite the high prevalence of NC alterations in anti-Ku patients major peripheral vascular complication such as ulcers are rare. Disclosure of Interest None Declared
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Research Interests: Immunology, Treatment Outcome, Medicine, Italy, Therapeutic drug monitoring, and 12 moreHumans, Systemic Lupus Erythematosus, Female, Follow-up studies, Antiphospholipid antibodies, Antibody, Adult, Combination drug therapy, Regimen, Glucocorticoids, Immunosuppressive Agents, and Medical and Health Sciences
ObjectivesThe outbreak of COVID-19 posed the issue of urgently identifying treatment strategies. Colchicine was considered for this purpose based on well-recognised anti-inflammatory effects and potential antiviral properties. In the... more
ObjectivesThe outbreak of COVID-19 posed the issue of urgently identifying treatment strategies. Colchicine was considered for this purpose based on well-recognised anti-inflammatory effects and potential antiviral properties. In the present study, colchicine was proposed to patients with COVID-19, and its effects compared with ‘standard-of-care’ (SoC).MethodsIn the public hospital of Esine, northern Italy, 140 consecutive inpatients, with virologically and radiographically confirmed COVID-19 admitted in the period 5–19 March 2020, were treated with ‘SoC’ (hydroxychloroquine and/or intravenous dexamethasone; and/or lopinavir/ritonavir). They were compared with 122 consecutive inpatients, admitted between 19 March and 5 April 2020, treated with colchicine (1 mg/day) and SoC (antiviral drugs were stopped before colchicine, due to potential interaction).ResultsPatients treated with colchicine had a better survival rate as compared with SoC at 21 days of follow-up (84.2% (SE=3.3%) vs 63...
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BackgroundCritically ill patients with coronavirus disease 2019 (COVID-19) have a profound hypercoagulable state and often develop coagulopathy which leads to organ failure and death. Because of a prolonged activated... more
BackgroundCritically ill patients with coronavirus disease 2019 (COVID-19) have a profound hypercoagulable state and often develop coagulopathy which leads to organ failure and death. Because of a prolonged activated partial-thromboplastin time (aPTT), a relationship with anti-phospholipid antibodies (aPL) has been proposed, but results are controversial. Functional assays for aPL (i.e., lupus anticoagulant) can be influenced by concomitant anticoagulation and/or high levels of C reactive protein. The presence of anti-cardiolipin (aCL), anti-beta2-glycoprotein I (anti-β2GPI) and anti-phosphatidylserine/prothrombin (aPS/PT) antibodies was not investigated systematically. Epitope specificity of anti-β2GPI antibodies was not reported.ObjectiveTo evaluate the prevalence and the clinical association of aPL in a large cohort of COVID-19 patients, and to characterize the epitope specificity of anti-β2GPI antibodies.MethodsELISA and chemiluminescence assays were used to test 122 sera of pat...
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Systemic sclerosis (SSc) is a rare systemic autoimmune disease, characterized by the presence of three main actors: vasculopathy, immune activation, and fibrosis. This pathologic process is then translated in a clinical picture with great... more
Systemic sclerosis (SSc) is a rare systemic autoimmune disease, characterized by the presence of three main actors: vasculopathy, immune activation, and fibrosis. This pathologic process is then translated in a clinical picture with great variability among different patients in terms of type of organ involvement, disease severity and prognosis. This heterogeneity is a main feature of SSc, which, in addition to the presence of early phases of the disease characterized by mild symptoms, can explain the high difficulty in establishing classification criteria, and in defining patients’ subsets and disease outcomes. The definition of disease outcomes is particularly relevant in the setting of clinical trials, where the aim is to provide reliable endpoints, able to measure the magnitude of the efficacy of a certain drug or intervention. For this reason, in the last years, increasing efforts have been done to design measures of disease activity, damage, severity, and response to treatment,...
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Belimumab (BEL) is a monoclonal antibody approved for SLE treatment but few data are available about its use before or during pregnancy.Our study aims to describe pregnancies in SLE patients who have discontinued BEL before conception, at... more
Belimumab (BEL) is a monoclonal antibody approved for SLE treatment but few data are available about its use before or during pregnancy.Our study aims to describe pregnancies in SLE patients who have discontinued BEL before conception, at positive pregnancy test or during pregnancy.Data from prospectively-followed pregnancies (2014-2020) in SLE patients treated with BEL in 3 Italian centers where retrospectively collected, focusing on maternal disease activity, obstetric and neonatal outcome. Continuous data are expressed as median [min-max].Thirteen SLE pregnancies were analyzed (median age at conception 32 [24-41] years; 77% spontaneous, 69% primigravidae). All patients had positive ANA and anti-dsDNA antibodies; 4 had anti-Ro antibodies (31%); 4 had anti-phospholipid antibodies (aPL; 1 single, 2 double and 1 triple positivity). Seven patients (54%) had a history of lupus nephritis (LN); 2 patients (15%) had a concomitant diagnosis of antiphospholipid syndrome (1 thrombotic-APS an...
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Objective To evaluate clinical associations of anti-PM/Scl antibodies in patients with SSc in a multicentre international cohort, with particular focus on unresolved issues, including scleroderma renal crisis (RC), malignancies, and... more
Objective To evaluate clinical associations of anti-PM/Scl antibodies in patients with SSc in a multicentre international cohort, with particular focus on unresolved issues, including scleroderma renal crisis (RC), malignancies, and functional outcome of interstitial lung disease (ILD). Methods (1) Analysis of SSc patients from the EUSTAR database: 144 anti-PM/Scl+ without SSc-specific autoantibodies were compared with 7202 anti-PM/Scl−, and then to 155 anti-Pm/Scl+ with SSc-specific antibodies. (2) Case–control study: additional data were collected for 165 anti-PM/Scl+ SSc patients (85 from the EUSTAR registry) and compared with 257 anti-PM/Scl− SSc controls, matched for sex, cutaneous subset, disease duration and age at SSc onset. Results Patients with isolated anti-PM/Scl+, as compared with anti-Pm/Scl−, had higher frequency of muscle involvement, ILD, calcinosis and cutaneous signs of DM, but similar frequency of SRC and malignancies (either synchronous with SSc onset or not). T...
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Joint Bone Spine - In Press.Proof corrected by the author Available online since samedi 28 juin 2014
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50 Autoimmune Polyendocrine Syndromes Angela Tincani, Angela Ceribelli, Ilaria Cavazzana, Franco Franceschini, Alberto Sulli and Maurizio Cutolo Abstract ... polyendocrine syndrome Type 1. J Clin Endocrinol Metab 88, 1442–4. Huang, W.,... more
50 Autoimmune Polyendocrine Syndromes Angela Tincani, Angela Ceribelli, Ilaria Cavazzana, Franco Franceschini, Alberto Sulli and Maurizio Cutolo Abstract ... polyendocrine syndrome Type 1. J Clin Endocrinol Metab 88, 1442–4. Huang, W., Connor, E., Dela Rosa, T., Muir, A ...
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Anti-Ro/SSA antibodies are associated with neonatal lupus but are also considered a possible cause for unexplainedpregnancy loss and adverse pregnancy outcome. In a large multicentres cohort study we have prospectivelyfollowed 100... more
Anti-Ro/SSA antibodies are associated with neonatal lupus but are also considered a possible cause for unexplainedpregnancy loss and adverse pregnancy outcome. In a large multicentres cohort study we have prospectivelyfollowed 100 anti-Ro/SSA positivewomen (53 systemic lupus erythematosus (SLE)) during their 122 pregnancies and 107 anti-Ro/SSA negative women (58 SLE) (140 pregnancies).Anti-Ro/SSA antibodies were tested by immunoblot and counterimmunoelectrophoresis. Mean gestational age at delivery (38 vs 37.9 weeks), prevalence of pregnancy loss (9.9 vs 18.6%), preterm birth (21.3 vs 13.9%), cesarean sections (49.2 vs 53.4%), premature rupture of membranes(4.9 vs 8.1%), preeclampsia(6.6 vs 8.1%), intrauterinegrowth retardation(0 vs 2.3%) and newborns small for gestationalage (11.5 vs 5.8%) were similar in anti-Ro/SSA positive and negative SLE mothers; findings were similar in non-SLE women. Two cases of congenital heart block were observed out of 100 anti-Ro/SSA positive women. In ...
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To evaluate the efficacy of hydroxychloroquine (HCQ) and quinacrine (Qn) association, at two different dosages, in treatment of lupus skin lesions not responding to HCQ alone. Thirty-four patients, affected by cutaneous and systemic lupus... more
To evaluate the efficacy of hydroxychloroquine (HCQ) and quinacrine (Qn) association, at two different dosages, in treatment of lupus skin lesions not responding to HCQ alone. Thirty-four patients, affected by cutaneous and systemic lupus erythematosus, were retrospectively analysed. They were treated by HCQ (5 mg/Kg/qd) and Qn with two regimens: 100 mg/qd (29 cases) and 50 mg/qd (5 cases). Discoid lupus erythematosus (19 cases), acute malar rash (6 cases), chilblain lupus (4 cases) showed a significant improvement with combination therapy ( P = 0.009, P = 0.019, and P = 0.04, respectively). Ten patients with subacute cutaneous lupus showed a partial response, whereas lupus profundus didn’t improve. The same overall response rate was recorded comparing two Qn regimens, but subjects taking 100 mg/qd improved more rapidly than the others ( P = 0.001). Ten patients developed side effects, mainly represented by skin yellowish discolouration. Depression and severe headache with nausea, w...
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Objective.To analyze the prevalence, associations, and fine specificity of autoantibodies to primary biliary cirrhosis (PBC)-associated antigens (MIT3, Sp100, and gp210) in a cohort of Italian patients with systemic sclerosis... more
Objective.To analyze the prevalence, associations, and fine specificity of autoantibodies to primary biliary cirrhosis (PBC)-associated antigens (MIT3, Sp100, and gp210) in a cohort of Italian patients with systemic sclerosis (SSc).Methods.Sera samples from 201 patients with SSc were tested for antibodies to MIT3, gp210, and Sp100 by ELISA (the PBC screen). Anti-MIT3-positive sera were studied for IgG or IgA isotypes. All sera were analyzed by indirect immunofluorescence on HEp-2 cells and on rodent kidney/stomach/liver tissue sections in order to detect antinuclear and antimitochondrial antibodies (AMA). SSc was selected by American College of Rheumatology criteria and classified based on LeRoy’s criteria.Results.Forty-three (21.4%) sera samples were positive for PBC screen antibodies. Anti-MIT3 antibodies were detected in 36 samples, anti-Sp100 in 5, and anti-gp210 in 1 sample. The other 3 PBC screen-positive samples showed no specificity for the single antigens. PBC screen-positi...
Research Interests: Rheumatology, Immunology, Gastroenterology, Medicine, Italy, and 15 moreHumans, Internal Medicine, Female, Male, The, Clinical Sciences, Aged, Middle Aged, Adult, Public health systems and services research, Sensitivity and Specificity, Primary Biliary Cirrhosis, Predictive value of tests, Autoantibodies, and Autoantigens
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Primary anti-phospholipid syndrome (PAPS) is an autoimmune condition defined by anti-phospholipid antibodies (aPL) and thrombotic or obstetric events. Some PAPS can evolve into systemic lupus erythematosus (SLE) during follow-up. Few... more
Primary anti-phospholipid syndrome (PAPS) is an autoimmune condition defined by anti-phospholipid antibodies (aPL) and thrombotic or obstetric events. Some PAPS can evolve into systemic lupus erythematosus (SLE) during follow-up. Few studies systematically examined lupus autoantibodies and their clinical significance in PAPS. The aim of our study is to analyze the clinical and laboratory correlations with lupus-related autoantibodies, detected by immunoprecipitation (IP), a technique not yet systematically applied to investigate autoantibodies in this condition. Sera from 52 PAPS patients were screened by indirect immunofluorescence (IIF) antinuclear antibodies (ANA), IP of ³⁵S-labeled K562 cell extract, and ELISA [anti-Argonaute2 (Ago2, Su), 60kRo, 52kRo, La, dsDNA)]. Anti-Ago2/Su positive sera were also tested for anti-GW bodies (GWBs) by IIF double staining, using rabbit anti-Rck/p54 serum. First, 56% of PAPS patients (29/52) were ANA positive, mainly with speckled pattern. Anti-Ago2/Su antibodies were found in 13% (7/52), anti-Ro/SSA in 10% (5/52), anti-La in one case. The clinical profile of patients did not seem to be related to the presence of these antibody specificities. However, levels of IgG anti-β2 glycoprotein I antibodies were lower in anti-Ago2/Su positive patients (p = 0.02). None of anti-Ago2/Su or -Ro patients developed SLE during a 2-year follow-up. Ago2 is a key component of GWBs, however, only 1/7 anti-Ago2/Su serum showed a typical cytoplasmic GWBs staining. Anti-Ago2/Su and -Ro antibodies are the two autoantibodies detected by IP in our PAPS cohort. Clarifying why Ago2/Su and Ro are specific targets of autoimmunity may help to understand the mechanisms of autoantibody production.
Research Interests: Immunology, Autoimmunity, Medicine, Dogs, Humans, and 15 moreSystemic Lupus Erythematosus, Mice, Female, Animals, Male, Young Adult, Immunoprecipitation, Primary Antiphospholipid Syndrome, Middle Aged, Adult, Rabbits, Ribonucleoproteins, Enzyme Linked Immunosorbent Assay, Autoantibodies, and Antiphospholipid Syndrome
We retrospectively compared disease activity, treatment, clinical and laboratory features, and rate of mortality of 535 SLE patients with adult and late disease onset. patients were divided into two groups based on the onset of the... more
We retrospectively compared disease activity, treatment, clinical and laboratory features, and rate of mortality of 535 SLE patients with adult and late disease onset. patients were divided into two groups based on the onset of the disease before or after 50 years of age. Clinical data were collected from medical reports. Disease activity was measured by ECLAM score. Parameters were compared by χ²-test, Fisher&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s test, Student&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s t or the Mann-Whitney test. Forty patients (7.5%) were included in the late SLE onset group (group A), while 495 (92.5%) in the adult SLE onset group (group B). Sicca symptoms were more frequent in group A (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0008), while glomerulonephritis (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0069), reduced C3 (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0006) and low C3 (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.00002) and C4 levels (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0006) were more prevalent in group B. Twenty-two deaths (4.3%) were recorded: 14 (2.8%) in group B and 8 (20%) in group A. Deaths were mainly due to infections in group B (28.5%) and cardiovascular events in group A (50%). A lower use of HCQ and LDA were recorded in deceased versus living patients (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.0001 and 0.0166, respectively), while a higher ECLAM score was measured at onset in dead versus living patients (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.048). Late onset SLE occurred in 7.5% of patients and it was associated with sicca symptoms. The use of HCQ and LDA is positively correlated with survival. Death in late onset SLE occurred more frequently for cardiovascular involvement. Higher disease activity at onset of the disease might represent a poor prognostic factor for death in adult onset.
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Research Interests: Rheumatology, Immunology, Biology, Medicine, Western blotting, and 15 moreHumans, Systemic Lupus Erythematosus, Autoimmune diseases, Female, Male, Arthritis, Immunoprecipitation, Clinical Sciences, Middle Aged, Fluorescent Antibody Technique, Adult, Public health systems and services research, Allergy and Immunology, Autoantibodies, and Polymyositis
To determine the prevalence of anti-Ku antibodies in 625 patients with systemic sclerosis (SSc) from six European rheumatological centres and to evaluate their clinical and serological characteristics. Sera of 625 consecutive patients... more
To determine the prevalence of anti-Ku antibodies in 625 patients with systemic sclerosis (SSc) from six European rheumatological centres and to evaluate their clinical and serological characteristics. Sera of 625 consecutive patients with either limited cutaneous or diffuse cutaneous SSc were tested for antibodies to Ku antigen together with other extractable nuclear antigens by counterimmunoelectrophoresis. A case-control design with calculation of bootstrap 95% confidence intervals derived from anti-Ku negative control patients was used to evaluate clinical associations of anti-Ku antibodies. Sera from anti-Ku positive patients with SSc and a control group were additionally tested by immunofluorescence on Hep-2 cell substrates and line immunoassay. Anti-Ku antibodies were found in the sera of 14/625 (2.2%) patients with SSc. Of 14 anti-Ku positive patients with SSc, 10 had no other anti-extractable nuclear antigen (ENA) antibodies detected by counterimmunoelectrophoresis. Using a case-control study design, anti-Ku antibodies were significantly associated with musculoskeletal manifestations such as clinical markers of myositis, arthritis and joint contractures. In addition, a significant negative correlation of anti-Ku antibodies was found with vascular manifestation such as fingertip ulcers and teleangiectasias. There was a striking absence of anti-centromere antibodies as well as anti- polymyositis (PM)/scleroderma (Scl) antibodies in patients that were anti-Ku positive. As expected, anti-Scl70 and punctate nucleolar immunofluorescence patterns were present only in single cases. This is the largest cohort to date focusing on the prevalence of anti-Ku antibodies in patients with SSc. The case-control approach was able to demonstrate a clinically distinct subset of anti-Ku positive patients with SSc with only relative clinical differences in skeletal features. However, the notable exceptions were signs of myositis. This shows the importance of anti-Ku antibody detection for the prediction of this specific clinical subset.
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Objective.To analyze clinical and serological characteristics of subjects with scleroderma renal crisis (SRC) in Italian patients with systemic sclerosis (SSc).Methods.A retrospective analysis of medical records from 9 Italian... more
Objective.To analyze clinical and serological characteristics of subjects with scleroderma renal crisis (SRC) in Italian patients with systemic sclerosis (SSc).Methods.A retrospective analysis of medical records from 9 Italian rheumatologic referral centers was carried out. All patients with SRC and an available serum sample at the time of crisis were included. Antinuclear antibodies (ANA) by indirect immunofluorescence, anti-topoisomerase (topo) I by enzyme-linked assay (ELISA), anti-RNA polymerases (RNAP) by ELISA for the subunit III, and immunoprecipitation (IP) were performed.Results.Forty-six cases (38 female; 40 diffuse cutaneous SSc) were identified. Mean age at SSc and SRC onset was 52.8 years ± 13.2 and 55.4 years ± 11.8, respectively. ANA were present in 44 patients (96%). Anti-topo I antibodies were detected in 30 (65%), anti-RNAP I–III in 7 (15%). No differences emerged between these 2 groups for their main clinical characteristics. The proportion of patients in the anti...
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... elettrocardiografica, e cioè il prolun-gamento del QT (corretto secondo la formula di Bazzett (>440msec), è stata recentemente segna-lata in bimbi nati da madri ... 16. Alarcon-Segovia D, Cardiel MH: Comparison between 3 diagnostic... more
... elettrocardiografica, e cioè il prolun-gamento del QT (corretto secondo la formula di Bazzett (>440msec), è stata recentemente segna-lata in bimbi nati da madri ... 16. Alarcon-Segovia D, Cardiel MH: Comparison between 3 diagnostic criteria for mixed connective tissue disea-se ...
Takayasu Arteritis (TAK) is a large-vessel vasculitis that preferentially involves the aorta and its primary branches. Cardiac involvement is frequent in TAK and is a major determinant of the patient's outcome. Glucocorticoids (GC)... more
Takayasu Arteritis (TAK) is a large-vessel vasculitis that preferentially involves the aorta and its primary branches. Cardiac involvement is frequent in TAK and is a major determinant of the patient's outcome. Glucocorticoids (GC) are the mainstay of therapy for TAK, with high doses of GC effective to induce remission. However, relapses are common and lead to repeated and prolonged GC treatments with high risk of related adverse events. Potential GC toxicity is a major concern, especially because patients with TAK are young and need to be treated for several years, often for the whole life. Conventional immunosuppressive drugs are used in patients with severe manifestations but present some limitations. New therapeutic approaches are needed for patients with refractory disease or contraindications to conventional therapies. Fortunately, major progress has been made in understanding TAK pathogenesis, leading to the development of targeted biotherapies. In particular, IL-6 and TN...
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The identification of anti-NXP2 antibodies is considered a serological marker of dermatomyositis (DM), with calcinosis, severe myositis and, in some reports, with cancer. Historically, these associations with anti-NXP2 antibodies have... more
The identification of anti-NXP2 antibodies is considered a serological marker of dermatomyositis (DM), with calcinosis, severe myositis and, in some reports, with cancer. Historically, these associations with anti-NXP2 antibodies have been detected by immunoprecipitation (IP), but in the last few years commercial immunoblotting assays have been released. The aim of this collaborative project was to analyse the clinical features associated to anti-NXP2 antibodies, both with commercial line blot (LB) and IP. Myositis-specific and myositis-associated autoantibodies were detected in single centres by commercial line blot (LB); available sera were evaluated in a single centre by protein and RNA immunoprecipitation (IP), and IP-Western blot. Sixty patients anti-NXP2+ (NXP2+) positive by LB were compared with 211 patients anti-NXP2 negative with idiopathic inflammatory myositis (IIM). NXP2+ showed a younger age at IIM onset (p = 0.0014), more frequent diagnosis of dermatomyositis (p = 0.02...
Research Interests: Immunology and Medicine
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Background. Critically ill patients with coronavirus disease 2019 (COVID-19) have a profound hypercoagulable state and often develop coagulopathy which leads to organ failure and death. Because of a prolonged activated... more
Background. Critically ill patients with coronavirus disease 2019 (COVID-19) have a profound hypercoagulable state and often develop coagulopathy which leads to organ failure and death. Because of a prolonged activated partial-thromboplastin time (aPTT), a relationship with anti-phospholipid antibodies (aPL) has been proposed, but results are controversial. Functional assays for aPL (i.e., lupus anticoagulant) can be influenced by concomitant anticoagulation and/or high levels of C reactive protein. The presence of anti-cardiolipin (aCL), anti-beta2-glycoprotein I (anti-{beta}2GPI and anti-phosphatidylserine/prothrombin (aPS/PT) antibodies was not investigated systematically. Epitope specificity of anti-{beta}2GPI antibodies was not reported. Aim. To evaluate the prevalence and the clinical association of aPL in a large cohort of COVID-19 patients, and to characterize the epitope specificity of anti-{beta}2GPI antibodies. Methods. ELISA and chemiluminescence assays were used to test...
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OBJECTIVES Patients with systemic lupus erythematosus (SLE) are prone to hypo-vitaminosis D because of their photosensitivity. Vitamin D (vit.D) has beneficial effects not only on bone metabolism but also on the function of the immune... more
OBJECTIVES Patients with systemic lupus erythematosus (SLE) are prone to hypo-vitaminosis D because of their photosensitivity. Vitamin D (vit.D) has beneficial effects not only on bone metabolism but also on the function of the immune system. The relationship between SLE disease activity and vit.D status is controversial and little is known on the effects of current supplementation strategies given for osteoporosis in raising vit.D levels. METHODS Vit.D levels were measured longitudinally in 50 SLE patients from Northern Italy at two time-points (winter and summer) during disease remission. Thirty patients were also evaluated during a flare. As controls, 170 healthy donors were enrolled. All the samples were analysed for 25-OH vit.D levels by a chemiluminescence assay (DiaSorin SpA, Italy). RESULTS SLE patients had lower vit.D levels than controls in the summer (median 29.4 vs. 39.2 ng/ml, p=0.005) but not in the winter (26.4 vs. 21.6 ng/ml). During wintertime, 36 SLE patients were ...
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GCA is the most frequent systemic vasculitis in patients older than 50 years involving medium-sized and large arteries. On July 2019 EULAR published its updated recommendations for the management of large vessel vasculitis, including... more
GCA is the most frequent systemic vasculitis in patients older than 50 years involving medium-sized and large arteries. On July 2019 EULAR published its updated recommendations for the management of large vessel vasculitis, including GCA.To analyze how the application of the updated EULAR recommendations changed clinical practice in GCA patients in our Hospital.All patients with a new diagnosis of GCA between January 1st, 2018 and December 31st, 2020 were enrolled in this study. Two cohorts were analyzed: patients who received GCA diagnosis in the eighteen months before EULAR recommendations publication (between January 1st, 2018 and June 30th, 2019: cohort A) and patients who received GCA diagnosis in the following eighteen months (between July 1st, 2019 and December 31st, 2020: cohort B). Data are expressed as median (IQR).70 patients were enrolled in the study (F: 47, M: 23, age: 76 (69-79) years): 39 patients in cohort A, 31 in cohort B. Table 1 summarize main clinical features ...
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Background: Giant Cells Arteritis (GCA) is the most common primary vasculitis in adults and usually occurs in patients older than 50 years. Epidemiological studies shown a higher prevalence of the disease in women compared to man.... more
Background: Giant Cells Arteritis (GCA) is the most common primary vasculitis in adults and usually occurs in patients older than 50 years. Epidemiological studies shown a higher prevalence of the disease in women compared to man. However, differences in clinical presentation between men and women have not been demonstrated, even if some distinctions have been suggested (1,2). Objectives: The purpose of the present study is to analyze differences in the clinical presentation of GCA according to sex. Methods: We collected retrospectively clinical data of a monocentric cohort of 100 consecutive GCA patients. Mann Whitney test was used to compare continuous variables, while Chi-square test and Fisher’s exact test were applied for comparison between qualitative variables. Results: One-hundred patients with a clinical diagnosis of GCA were enrolled in the study (68 women, 32 men). In all patients the diagnosis of vasculitis was histologically and/or radiologically confirmed. Main clinica...
SUMMARY Objective: to analyse efficacy and safety of anti-TNFα treatment in 17 patients with rheumatoid arthritis (AR) and anti-Ro antibodies, in order to detect difference in clinical and immunological response. Methods: 322 patients,... more
SUMMARY Objective: to analyse efficacy and safety of anti-TNFα treatment in 17 patients with rheumatoid arthritis (AR) and anti-Ro antibodies, in order to detect difference in clinical and immunological response. Methods: 322 patients, affected by RA and treated with anti-TNFα drugs, were considered, searching every 6-12 months ANA, anti-dsDNA and anti-ENA antibodies. Seventeen were anti-Ro positive and 305 anti-Ro negative before starting treatment. Results: anti-Ro positive subjects showed active arthritis at baseline (mean DAS: 5), with frequent extra-articular features, such as ocular and oral sicca symptoms. They showed rapid and stable improvement during the treatment, without significant difference compared to anti-Ro negative group. A good clinical Eular response was shown in 46% of anti-Ro negative subjects, steady stable during time. On the contrary, fewer anti-Ro positive patients seem to be “good” responders. RA remission (DAS <1,6) was achieved in 9-25% of anti-Ro po...
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Objectives Serum anti-dsDNA and anti-nucleosome IgGs have been proposed as signatures for SLE and LN in limited numbers of patients. We sought to show higher sensitivity and specificity of the same antibodies with the IgG2 isotype and... more
Objectives Serum anti-dsDNA and anti-nucleosome IgGs have been proposed as signatures for SLE and LN in limited numbers of patients. We sought to show higher sensitivity and specificity of the same antibodies with the IgG2 isotype and included IgG2 antibodies vs specific intracellular antigens in the analysis. Methods A total of 1052 SLE patients with (n = 479) and without (n = 573) LN, recruited at different times from the beginning of symptoms, were included in the study. Patients with primary APS (PAPS, n = 24), RA (RA, n = 24) and UCTD (UCTD, n = 96) were analysed for comparison. Anti-nucleosome (dsDNA, Histone2A, Histone3), anti-intracellular antigens (ENO1), anti-annexin A1 and anti-C1q IgG2 were determined by non-commercial techniques. Results The presence in the serum of the IgG2 panel was highly discriminatory for SLE/LN vs healthy subjects. Serum levels of anti-dsDNA and anti-C1q IgG2 were more sensitive than those of IgGs (Farr radioimmunoassay/commercial assays) in ident...
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Objective: The long-term outcome of children born to SLE mothers still represents a controversial topic in literature, with some studies reporting a possible increased prevalence of different neurologic and psychiatric diseases (NPD),... more
Objective: The long-term outcome of children born to SLE mothers still represents a controversial topic in literature, with some studies reporting a possible increased prevalence of different neurologic and psychiatric diseases (NPD), including neurodevelopmental disorders (ND), and in particular learning disorders (LD). Different risk factors have been advocated, such as the in utero exposure to auto-antibodies and drugs, particularly Azathioprine (AZA).Methods: A case-control study was designed to compare pregnancies treated with AZA (cases) with those not treated with AZA (controls). All the pregnancies had been prospectively followed in two Italian centers. The match was based upon renal involvement, antiphospholipid (aPL) status, maternal age at pregnancy (±5 years) and child’s age at the time of the study (±2 years). SLE mothers were interviewed by a telephone survey, particularly focused on the presence of a certified NPD in their children ≥6 years of age.Results: Twenty-seve...
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Antisynthetase syndrome (ASSD) is a rare clinical condition that is characterized by the occurrence of a classic clinical triad, encompassing myositis, arthritis, and interstitial lung disease (ILD), along with specific autoantibodies... more
Antisynthetase syndrome (ASSD) is a rare clinical condition that is characterized by the occurrence of a classic clinical triad, encompassing myositis, arthritis, and interstitial lung disease (ILD), along with specific autoantibodies that are addressed to different aminoacyl tRNA synthetases (ARS). Until now, it has been unknown whether the presence of a different ARS might affect the clinical presentation, evolution, and outcome of ASSD. In this study, we retrospectively recorded the time of onset, characteristics, clustering of triad findings, and survival of 828 ASSD patients (593 anti-Jo1, 95 anti-PL7, 84 anti-PL12, 38 anti-EJ, and 18 anti-OJ), referring to AENEAS (American and European NEtwork of Antisynthetase Syndrome) collaborative group’s cohort. Comparisons were performed first between all ARS cases and then, in the case of significance, while using anti-Jo1 positive patients as the reference group. The characteristics of triad findings were similar and the onset mainly b...
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ABSTRACT Background Polymyositis/dermatomyositis (PM/DM) are autoimmune diseases characterized by skin and muscle inflammation, internal organs involvement and presence of myositis-specific antibodies (MSAs). In recent years, new MSAs... more
ABSTRACT Background Polymyositis/dermatomyositis (PM/DM) are autoimmune diseases characterized by skin and muscle inflammation, internal organs involvement and presence of myositis-specific antibodies (MSAs). In recent years, new MSAs have been identified in association with specific clinical features, as anti-CADM140/MDA5 (melanoma differentiation-associated gene 5), -TIF1gamma/alpha (p155/140) and -MJ/NXP-2. Among these, anti-MDA5 antibodies are strongly associated with clinically amyopathic DM (CADM), rapidly progressive interstitial lung disease (RPILD), severe skin manifestations and worse prognosis. In most cases, anti-MDA5 antibodies are detected in Eastern Asian (Japan, Korea, and China) cohorts, with the exception of only one American study. Objectives The aim of our work is to identify anti-MDA5 antibodies and their clinical associations in a cohort of Italian (European Caucasian) patients with PM/DM. Methods Sera of 79 consecutive adult Italian (European Caucasian) patients with PM/DM were collected in the outpatient clinic of the Rheumatology Unit in Brescia (Spedali Civili) in 2009-2012. The criteria of Bohan and Peter for PM/DM and the criteria of Sontheimer for CADM were used to establish the diagnosis. All MSAa were characterized by immunoprecipitation (IP) of 35S-methionin radiolabeled K562 (human erythroleukemia) cell extract except anti-MDA5; anti-MDA5 was confirmed by ELISA (recombinant MDA5 protein and 1:250 diluted sera) and immunoprecipitation-Western Blot (IP-WB) using rabbit anti-MDA5 antibodies. Clinical information were obtained from clinical charts. Results Anti-MDA5 antibodies were identified in 6/79 (8%) PM/DM cases, and they were the second most common specificity after anti-MJ (8/79; 10%); 23% (8/35) DM and 20% (16/79) in the whole PM/DM cohort still did not have known myositis antibodies. Anti-MDA5 was found in 17% (6/35) in DM and 5/6 cases were CADM, indicating strong association between anti-MDA5 and CADM. The clinical features of 6 anti-MDA5 (+) DM cases were analyzed vs 29 anti-MDA5 (-) DM. Compared to anti-MDA5 (-) DM, anti-MDA5 (+) have severe skin disease, mainly represented by skin ulcers (digit pulp/periungual and Gottron’s lesions) and heliotrope rash (67% vs 55%). ILD is more common in the anti-MDA5 (+) DM patients (50%) vs anti-MDA5 (-) (14%): two patients underwent cyclophosphamide therapy, and one died due to RPILD. One also developed subcutaneous emphysema with pneumomediastinum. 83% of anti-MDA5 (+) had CADM (vs 17% CADM in anti-MDA5 (-); p=0.004). Only one anti-MDA5 (+) patient had moderate increase of CPK and muscle weakness. No significant association was identified between anti-MDA5 antibodies and other DM symptoms, such as Raynaud’s phenomenon, oral ulcers, calcinosis and higher risk of cancer-associated DM. None of anti-MDA5 (+) cases had other MSAs. Conclusions Our study is the first report on anti-MDA5 antibodies in adult Italian (European Caucasian) patients with PM/DM. Anti-MDA5 antibodies were detected in 8% (6/79) Italian PM/DM, all affected by DM. Anti-MDA5 antibodies are not specific for Asian patients but they are also common in Italian patients with CADM. Disclosure of Interest: None Declared
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Background and objectivesAntimitochondrial (AMA) is considered the serological hallmark of primary biliary cirrhosis (PBC). Other autoantibodies recognising nuclear dots (Sp100) and nuclear pore complex proteins (gp-210) are associated to... more
Background and objectivesAntimitochondrial (AMA) is considered the serological hallmark of primary biliary cirrhosis (PBC). Other autoantibodies recognising nuclear dots (Sp100) and nuclear pore complex proteins (gp-210) are associated to severe PBC, but they are found by less available methods. An ELISA with a combination of three mitochondrial antigens (MIT3), Sp100 and gp-210 has been recently developed. The aim of our
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Antiphospholipid antibodies (aPL) can induce fetal loss in experimental animal models. Human studies did find hypocomplementemia associated with pregnancy complications in patients with antiphospholipid syndrome (APS), but these results... more
Antiphospholipid antibodies (aPL) can induce fetal loss in experimental animal models. Human studies did find hypocomplementemia associated with pregnancy complications in patients with antiphospholipid syndrome (APS), but these results are not unanimously confirmed. To investigate if the detection of low C3/C4 could be considered a risk factor for adverse pregnancy outcomes (APO) in APS and aPL carriers’ pregnancies we performed a multicenter study including 503 pregnancies from 11 Italian and 1 Russian centers. Data in women with APS and asymptomatic carriers with persistently positive aPL and preconception complement levels were available for 260 pregnancies. In pregnancies with low preconception C3/C4, a significantly higher prevalence of pregnancy losses was observed (p = 0.008). A subgroup analysis focusing on triple aPL-positive patients found that preconception low C3 and/or C4 levels were associated with an increased rate of pregnancy loss (p = 0.05). Our findings confirm t...
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The formation of neutrophil extracellular traps (NETs) is a strategy utilized by neutrophils for capturing infective agents. Extracellular traps consist in a physical net made of DNA and intracellular proteins externalized from... more
The formation of neutrophil extracellular traps (NETs) is a strategy utilized by neutrophils for capturing infective agents. Extracellular traps consist in a physical net made of DNA and intracellular proteins externalized from neutrophils, where bacteria and viruses are entrapped and killed by proteolysis. A complex series of events contributes to achieving NET formation: signaling from infectious triggers comes first, followed by decondensation of chromatin and extrusion of the nucleosome components (DNA, histones) from the nucleus and, after cell membrane breakdown, outside the cell. NETs are composed of either DNA or nucleosome proteins and hundreds of cytoplasm proteins, a part of which undergo post-translational modification during the steps leading to NETs. There is a thin balance between the production and the removal of circulating NETs from blood where digestion of DNA by circulating DNases 1 and IL3 has a critical role. A delay in NET removal may have consequences for aut...
Objective The aim of our study was to investigate clinical and histopathological findings in adult DM patients positive for anti-Mi2 (anti-Mi2+) antibodies compared with DM patients negative for anti-Mi2 (anti-Mi2–). Methods Clinical data... more
Objective The aim of our study was to investigate clinical and histopathological findings in adult DM patients positive for anti-Mi2 (anti-Mi2+) antibodies compared with DM patients negative for anti-Mi2 (anti-Mi2–). Methods Clinical data of adult DM patients, who fulfilled EULAR/ACR 2017 classification criteria, were gathered from electronic medical records of three tertiary Rheumatology Units. Histopathological study was carried out on 12 anti-Mi2+ and 14 anti-Mi2– muscle biopsies performed for diagnostic purpose. Nine biopsies from immune mediated necrotizing myopathy (IMNM) patients were used as control group. Results Twenty-two anti-Mi2+ DM [90.9% female, mean age 56.5 (15.7) years] were compared with 69 anti-Mi2– DM patients [71% female, mean age 52.4 (17) years]. Anti-Mi2+ patients presented higher levels of serum muscle enzymes than anti-Mi2– patients [median (IQR) creatine-kinase fold increment: 16 (7–37)vs 3.5 (1–9.9), P <0.001] before treatment initiation. Moreover, a ...
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Interstitial lung disease (ILD) represents one of the most severe extra-muscular features of idiopathic inflammatory myositis (IIM). We aimed to identify any clinical and serological predictors of ILD in a monocentric cohort of 165 IIM... more
Interstitial lung disease (ILD) represents one of the most severe extra-muscular features of idiopathic inflammatory myositis (IIM). We aimed to identify any clinical and serological predictors of ILD in a monocentric cohort of 165 IIM patients. ILD+ patients were defined as having restrictive impairment in lung function tests and signs of ILD at chest high-resolution computed tomography (HRCT). Available HRCT images were centralized and classified in different ILD patterns: non-specific interstitial pneumonia (NSIP), organizing pneumonia (OP), usual interstitial pneumonia-like (UIP), indeterminate for UIP, and interstitial lung abnormalities (ILA). Lung function test data were recorded at onset, at 1 and 5 years after ILD diagnosis. ILD was found in 52 IIM patients (31.5%): 46.2% was affected by anti-synthetase syndrome (ARS), 21% by polymyositis (PM), 19% by dermatomyositis (DM), and 13.5% by overlap myositis. Most of ILD+ showed NSIP (31.9%), OP (19%), indeterminate for UIP (19%)...
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Association of celiac disease (CD) with systemic autoimmune diseases (ADs) remains controversial. Awareness of CD in these patients is important to prevent complications, including lymphoproliferative disorders. We evaluated previously... more
Association of celiac disease (CD) with systemic autoimmune diseases (ADs) remains controversial. Awareness of CD in these patients is important to prevent complications, including lymphoproliferative disorders. We evaluated previously diagnosed CD prevalence in systemic lupus erythematosus (SLE), primary Sjögren’s syndrome (pSS) and systemic sclerosis (SSc) patients in comparison to 14,298 matched controls. All patients were screened for subclinical CD. Data from 1458 unselected consecutive SLE (580), pSS (354) and SSc (524) patients were collected. Previously biopsy-proven CD diagnosis and both CD- and AD-specific features were registered. All patients without previous CD were tested for IgA transglutaminase (TG). Anti-endomysium were tested in positive/borderline IgA TG. Duodenal biopsy was performed in IgA TG/endomysium+ to confirm CD. CD prevalence in AD was compared to that observed in 14,298 unselected sex- and age-matched adults who acted as controls. CD was more prevalent i...
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A high rate of left ventricular diastolic dysfunction (LVDD) has been described in rheumatoid arthritis (RA), compared with general population. A prospective study demonstrated that LVDD occurred in 24% in one year of follow-up in RA... more
A high rate of left ventricular diastolic dysfunction (LVDD) has been described in rheumatoid arthritis (RA), compared with general population. A prospective study demonstrated that LVDD occurred in 24% in one year of follow-up in RA patients without cardiac disease. Older age, higher systolic arterial pressure and lower E/A ratio are considered predictive factors. In addition, in RA, LVDD is known to be the only risk factor for the development of cardiovascular (CV) events, also in absence of classical CV risk factors. Some occasional reports suggest that early and aggressive treatment of RA could influence the evolution of LVDD and, accordingly, modify the rate of CV events. Therefore, a correct assessment of diastolic function should be considered of pivotal importance in the routine follow-up of RA patients.
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This review focuses on new clinical aspects of antiphospholipid syndrome (APS) in the last 5 years. The pathogenesis of APS is related to endothelial activation by mechanisms other than autoantibody-mediated massive coagulation. These... more
This review focuses on new clinical aspects of antiphospholipid syndrome (APS) in the last 5 years. The pathogenesis of APS is related to endothelial activation by mechanisms other than autoantibody-mediated massive coagulation. These include Toll-like receptors, the m-TORC pathway, and neutrophil activation, inducing an uncontrolled inflammatory cascade. Given these new pathogenetic hypotheses, the treatment of APS could be directed towards a fine balance between anticoagulation and immunomodulation. A hot topic is how to consider asymptomatic antiphospholipid (aPL) carriers, with or without systemic lupus erythematosus (SLE), during pregnancy, or during their life in general: to treat or not to treat? New findings on long-standing APS, regarding survival, comorbidities, and evolution in other autoimmune conditions, have become available, including new insights into aPL as potential risk factors for damage accrual in SLE and potential implications on neuropsychological involvement ...
To provide the most recent evidence on the overlap myositis. Several new evidences on the overlap myositides have recently emerged. Regarding the classical myositis associated antibodies, several contributions focused on a better... more
To provide the most recent evidence on the overlap myositis. Several new evidences on the overlap myositides have recently emerged. Regarding the classical myositis associated antibodies, several contributions focused on a better definition of the clinical associations and the disease course associated with these autoantibodies. Moreover, in the last years, new autoantibodies in idiopathic inflammatory myositis or other connective tissue diseases have been identified [namely anti-RuvBL1/2, poly-U-binding factor 60 kDa protein (PUF-60) and cytosolic 5'-nucleotidase 1A (NT5C1A)], and an increasing number of publications allow now to consider them as new myositis-associated antibodies with probably their own peculiar clinical profile. Overlap myositis is probably the largest subgroup within the idiopathic inflammatory myositis, with a prevalence that can reach 50% of all adult patients. The serological spectrum of overlap myositis has recently been enriched by the discovery of new ...
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The aim of the present study was to determine the prevalence of lower urinary tract symptoms (LUTS) in systemic sclerosis (SSc), to find specific risk factors and to assess their impact on quality of life (QoL). In a multi-centric study,... more
The aim of the present study was to determine the prevalence of lower urinary tract symptoms (LUTS) in systemic sclerosis (SSc), to find specific risk factors and to assess their impact on quality of life (QoL). In a multi-centric study, 334 patients completed a self-administered questionnaire on LUTS and QoL. LUTS were classified into three main categories: storage, voiding, and post micturition symptoms. Digestive symptoms burden was captured by a visual analogic scale, divided into five equal categories. Multivariable logistic regressions were performed to test association between risk factors and LUTS categories. Linear regression adjusted the association between LUTS and QoL. LUTS were recorded in 311 SSc patients (96.0%) and classified as severe in 120 (38.0%). The storage category of LUTS was the most prevalent (91.9%) followed by voiding (72.2%) and post micturition symptoms (49.8%). Risk factors identified in the multivariable models were higher than the median HAQ-DI (OR: ...
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To assess the prevalence and risk factors for endothelial dysfunction detected by peripheral artery tonometry in systemic lupus erythematosus patients with early disease without cardiovascular disease and risk factors. All the consecutive... more
To assess the prevalence and risk factors for endothelial dysfunction detected by peripheral artery tonometry in systemic lupus erythematosus patients with early disease without cardiovascular disease and risk factors. All the consecutive adult lupus patients, with a disease duration less than 5 years, seen in our Hospital from December 2014 to March 2016 were considered. We excluded patients with any history of cardiovascular disease or risk factors possibly affecting peripheral artery tonometry. Enrolled patients were matched for sex, age, body mass index and blood pressure with healthy controls with the same exclusion criteria. Patients and controls performed a transthoracic Doppler echocardiogram and an evaluation of endothelial function by peripheral artery tonometry. Twenty patients (100% female) with a median disease duration of 14 months (1-58), a mean age of 42±15 years and a mean age at diagnosis of 40±16 years were enrolled and matched with 20 controls. Peripheral artery ...
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Objective.To assess the longterm frequency of thrombotic recurrences, obstetrical complications, organ damage, severe comorbidities, and evolution toward connective tissue disease (CTD) in primary antiphospholipid syndrome... more
Objective.To assess the longterm frequency of thrombotic recurrences, obstetrical complications, organ damage, severe comorbidities, and evolution toward connective tissue disease (CTD) in primary antiphospholipid syndrome (PAPS).Methods.Medical records of patients with PAPS followed in 6 centers for ≥ 15 years were retrospectively reviewed.Results.One hundred fifteen patients were studied: 88% women, followed between 1983 and 2014 with a mean (± SD) age at diagnosis of 33 (± 10) years. During a median followup of 18 years (range 15–30), 50 patients (44%) had at least a thrombotic event for a total of 75 events and an annual incidence of 3.5%. Thromboses were more frequent in patients with previous thrombotic history (p = 0.002). A catastrophic antiphospholipid syndrome occurred in 6 patients (5%). The use of oral anticoagulants in patients with thrombotic onset did not appear to be protective against recurrences (p = 0.26). Fifty-two women had 87 pregnancies, successful in 78%. Twe...
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To evaluate if the timing of appearance with respect to disease onset may influence the arthritis presentation pattern in antisynthetase syndrome (ASSD). The patients were selected from a retrospective large international cohort of ASSD... more
To evaluate if the timing of appearance with respect to disease onset may influence the arthritis presentation pattern in antisynthetase syndrome (ASSD). The patients were selected from a retrospective large international cohort of ASSD patients regularly followed-up in centres referring to AENEAS collaborative group. Patients were eligible if they had an antisynthetase antibody testing positive in at least two determinations along with arthritis occurring either at ASSD onset (Group 1) or during the course of the disease (Group 2). 445 (70%; 334 females, 110 males, 1 transsexual) out of the 636 ASSD we collected had arthritis, in the majority of cases (367, 83%) from disease onset (Group 1). Patients belonging to Group 1 with respect to Group 2 had an arthritis more commonly polyarticular and symmetrical (p=0.015), IgM-Rheumatoid factor positive (p=0.035), erosions at hands and feet plain x-rays (p=0.036) and more commonly satisfying the 1987 revised classification criteria for rhe...
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Arthritis, myositis and interstitial lung disease (ILD) constitute the classic clinical triad of anti-synthetase syndrome (ASSD). These patients experience other accompanying features, such as Raynaud's phenomenon, fever or... more
Arthritis, myositis and interstitial lung disease (ILD) constitute the classic clinical triad of anti-synthetase syndrome (ASSD). These patients experience other accompanying features, such as Raynaud's phenomenon, fever or mechanic's hands. Most ASSD patients develop the complete triad during the follow-up. In the present study we aimed to determine whether the subsequent appearance of accompanying features may suggest the development of triad findings lacking at the onset in anti-Jo1 positive ASSD patients. Anti-Jo1 positive patients presenting with incomplete ASSD (no >2 classic triad features) were assessed. Clinical characteristics and clusters of disease manifestations were retrospectively collected and analyzed in a large international multicenter cohort of ASSD patients. 165 patients (123 women) with incomplete ASSD were identified. Ninety-five patients (57.5%) developed new classic triad manifestations after 15months median (IQR 9-51) and 40 (24%) developed new a...
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Morphea is a rare fibrosing skin disorder. Antinuclear antibodies (ANA), antihistone and rheumatoid factor are detected in high rate of morphea cases. Scleroderma-related antibodies are usually absent. 21 adult patients affected by... more
Morphea is a rare fibrosing skin disorder. Antinuclear antibodies (ANA), antihistone and rheumatoid factor are detected in high rate of morphea cases. Scleroderma-related antibodies are usually absent. 21 adult patients affected by morphea were examined at the time of enrollment and after 6 months with the assessment of clinical outcome measures of disease severity and damage. Healthy subjects were considered as normal controls while patients affected by systemic sclerosis and other connective tissue diseases (CTD) were considered as pathological controls. Serum samples from all the patients and controls were analysed for the detection of ANA, anti-nucleosome antibodies, anti-dsDNA and anti-ENA. Scleroderma-related autoantibodies were searched using a line-blot test. We enrolled 21 patients affected by morphea. ANA were found in 12 patients (57%). Anti-DNA and anti-nucleosome antibodies were negative in all cases. SSc-specific antibodies were found in 11 morphea patients (52.4%) and...
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We aimed to identify the possible clinical and laboratory predictors of calcinosis in a cohort of patients with a diagnosis of polymyositis (PM) and dermatomyositis (DM). We carried out a retrospective analysis of a cohort of myositis... more
We aimed to identify the possible clinical and laboratory predictors of calcinosis in a cohort of patients with a diagnosis of polymyositis (PM) and dermatomyositis (DM). We carried out a retrospective analysis of a cohort of myositis patients attending our clinic between January 2013 and May 2014. 74 patients (58 females, 16 males) with PM (30 cases), DM (30 cases), overlap syndrome (13 cases) and inclusion body myositis (1 case) were enrolled. Sixteen patients (21.6%) had calcinosis that occurred a mean of 43.7 months after diagnosis of PDM. At multivariate analysis, patients with calcinosis experienced longer follow-up duration (p=0.006), anti-PM/Scl (p=0.033) and anti-NXP2 (p=0.024) positivity compared to patients without calcinosis. Furthermore, anti-NXP-2 positive C+ showed a diffuse form of calcinosis from the beginning and lower frequency of respiratory tract involvement. No single drug or associations of drugs was found effective in the treatment of calcinosis. A longer fol...
Research Interests: Biomarkers, Japan, Multivariate Analysis, Humans, Female, and 15 moreMale, Risk factors, Clinical Sciences, Middle Aged, Calcinosis, Adult, Odds ratio, Retrospective Studies, Chi Square Distribution, Risk Factors, Dermatomyositis, DNA binding proteins, Logistic Models, Autoantibodies, and Polymyositis
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In an effort to find naturally occurring substances that reduce cholesterol by inhibiting 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR), statins were first discovered by Endo in 1972. With the widespread prescription and use of... more
In an effort to find naturally occurring substances that reduce cholesterol by inhibiting 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR), statins were first discovered by Endo in 1972. With the widespread prescription and use of statins to decrease morbidity from myocardial infarction and stroke, it was noted that approximately 5% of all statin users experienced muscle pain and weakness during treatment. In a smaller proportion of patients, the myopathy progressed to severe morbidity marked by proximal weakness and severe muscle wasting. Remarkably, Mammen and colleagues were the first to discover that the molecular target of statins, 3-hydroxy-3-methylglutaryl coenzyme A reductase (HMGCR), is an autoantibody target in patients that develop an immune-mediated necrotizing myopathy (IMNM). These observations have been confirmed in a number of studies but, until today, a multi-center, international study of IMNM, related idiopathic inflammatory myopathies (IIM), other auto-inflammatory conditions and controls has not been published. Accordingly, an international, multi-center study investigated the utility of anti-HMGCR antibodies in the diagnosis of statin-associated IMNM in comparison to different forms of IIM and controls. This study included samples from patients with different forms of IIM (n=1250) and patients with other diseases (n=656) that were collected from twelve sites and tested for anti-HMGCR antibodies by ELISA. This study confirmed that anti-HMGCR autoantibodies, when found in conjunction with statin use, characterize a subset of IIM who are older and have necrosis on muscle biopsy. Taken together, the data to date indicates that testing for anti-HMGCR antibodies is important in the differential diagnosis of IIM and might be considered for future classification criteria.
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To investigate effectiveness and safety of belimumab in patients with active systemic lupus erythematosus (SLE) in clinical practice setting. Sixty-seven patients with active SLE, mean age 39.3±10.2 years, from two Italian prospective... more
To investigate effectiveness and safety of belimumab in patients with active systemic lupus erythematosus (SLE) in clinical practice setting. Sixty-seven patients with active SLE, mean age 39.3±10.2 years, from two Italian prospective cohorts were treated with belimumab (10 mg/kg day 0, 14, 28, and then every 28 days) added to background therapy. SLEDAI-2K, SLICC-DI, DAS28, 24-hours proteinuria, CLASI (Cutaneous LE Disease Area and Severity Index) activity score, anti-dsDNA, C3, C4, and prednisone daily dose were recorded at baseline, month 3, 6, 9, 12, 18 and 24. Arthritis was subdivided into "classical" (CLP) and "rheumatoid-like"; skin manifestations into acute (ACLE), subacute (SCLE) and chronic. SLE flares, defined according to SLEDAI flare index, were calculated before and after belimumab initiation. Adverse events were carefully evaluated during treatment. Statistics were performed by SPSS package (version 21.0). Mean±SD follow-up was 16.2±9.5 months. Main...
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Research Interests: Immunology, Multivariate Analysis, Humans, Systemic Lupus Erythematosus, Female, and 15 moreMale, Infant, Clinical Sciences, Newborn Infant, Public health systems and services research, Biological markers, Disease Progression, Sensitivity and Specificity, Reference Values, Enzyme Linked Immunosorbent Assay, Logistic Models, Autoantibodies, Cohort Studies, Severity of Illness Index, and Child preschool
Patients with systemic lupus erythematosus (SLE) are prone to hypo-vitaminosis D because of their photosensitivity. Vitamin D (vit.D) has beneficial effects not only on bone metabolism but also on the function of the immune system. The... more
Patients with systemic lupus erythematosus (SLE) are prone to hypo-vitaminosis D because of their photosensitivity. Vitamin D (vit.D) has beneficial effects not only on bone metabolism but also on the function of the immune system. The relationship between SLE disease activity and vit.D status is controversial and little is known on the effects of current supplementation strategies given for osteoporosis in raising vit.D levels. Vit.D levels were measured longitudinally in 50 SLE patients from Northern Italy at two time-points (winter and summer) during disease remission. Thirty patients were also evaluated during a flare. As controls, 170 healthy donors were enrolled. All the samples were analysed for 25-OH vit.D levels by a chemiluminescence assay (DiaSorin SpA, Italy). SLE patients had lower vit.D levels than controls in the summer (median 29.4 vs. 39.2 ng/ml, p=0.005) but not in the winter (26.4 vs. 21.6 ng/ml). During wintertime, 36 SLE patients were supplemented with vit.D drops (n=24; 48%), vit.D+calcium tablets (n=12; 24%), while 14 (28%) received no supplementation. Patients on oral drops had significantly higher vit.D levels than patients on tablets. The median weekly dosage was higher for oral drops than for tablets (6250 vs. 4560 UI, p=0.009). Winter flares were associated with lower vit.D levels in comparison with remission during the same season for each patient (21.1 vs. 30 ng/ml). Current strategies of vit.D supplementation seem to be not sufficient for reaching an optimal vit.D status in Italian SLE patients. Vit.D and calcium tablets were less effective, probably because of lower vit.D content and poorer compliance. Vit.D insufficiency detected in the wintertime can be either a predisposing factor for flare or the consequence of the flare itself in SLE patients.
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Polymyositis/dermatomyositis (PM/DM) is an autoimmune disease characterised by skin and muscle inflammation, internal organ involvement and serum disease-specific autoantibodies. The recently identified anti-MDA5 (melanoma... more
Polymyositis/dermatomyositis (PM/DM) is an autoimmune disease characterised by skin and muscle inflammation, internal organ involvement and serum disease-specific autoantibodies. The recently identified anti-MDA5 (melanoma differentiation-associated gene 5) antibodies are associated with clinically amyopathic DM (CADM), rapidly progressive interstitial lung disease, severe skin manifestations, and poor prognosis. Our objective was to examine the clinical significance of anti-MDA5 antibodies in a cohort of European Caucasian patients with PM/DM, considering that data on anti-MDA5 serology are limited to Asian and US cohorts. Sera from 76 consecutive adult Italian patients with PM/DM were analysed by immunoprecipitation (IP) of 35S-methionine radiolabelled HeLa and K562 cell extracts, ELISA using recombinant MDA5 protein and IP-Western Blot using rabbit anti-MDA5 antibodies. Clinical associations of anti-MDA5 antibody positive patients were analysed. Anti-MDA5 antibodies were identified in 5/76 (7%) PM/DM cases and all 5 cases were CADM; anti-MDA5 was the second most common autoantibody in DM after anti-MJ/NXP-2, found in 24% of cases. Compared to 29 anti-MDA5 (-) DM, anti-MDA5 (+) patients have more typical DM skin disease (digit pulp/periungual lesions, Gottron&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s papules, heliotrope rash) (p=ns). Interstitial lung disease was observed in 3/5 anti-MDA5 (+) patients but only 14% of anti-MDA5 (-) cases (p=0.048). Our study on European patients with PM/DM confirms that anti-MDA5 antibodies are not uncommon. All anti-MDA5 (+) cases are affected by CADM with typical skin disease, while rapidly progressive pulmonary involvement was diagnosed only in one case. Further studies in larger cohorts are necessary to define the clinical significance of anti-MDA5 antibodies in European PM/DM.
Research Interests: Western blotting, Italy, Humans, Female, Male, and 15 morePhenotype, Immunoprecipitation, Clinical Sciences, Aged, Prevalence, Middle Aged, Adult, HeLa cells, Retrospective Studies, European Continental Ancestry Group, Biological markers, Dermatomyositis, Enzyme Linked Immunosorbent Assay, Predictive value of tests, and Autoantibodies
Research Interests: Immunology, Humans, Systemic Lupus Erythematosus, Female, Male, and 14 moreThe, Clinical Sciences, Aged, Middle Aged, Adult, Public health systems and services research, Retrospective Studies, Biological markers, Sensitivity and Specificity, DNA binding proteins, Autoantibodies, Immunoblotting, Polymyositis, and Connective Tissue Diseases
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SUMMARY Objective: To assess the prevalence of Congenital Heart Block (CHB) in newborns from anti Ro/SS-A antibodies pos- itive mothers affected by connective tissue diseases (CTD) and to evaluate the prevalence of other manifestations of... more
SUMMARY Objective: To assess the prevalence of Congenital Heart Block (CHB) in newborns from anti Ro/SS-A antibodies pos- itive mothers affected by connective tissue diseases (CTD) and to evaluate the prevalence of other manifestations of Neonatal Lupus (NL) and the electrocardiographic abnormalities. Methods: A prospective study was conducted on 100 anti Ro/SS-A positive mothers that were followed before and dur- ing their 118 pregnancies (4 twin pregnancies and 18 second pregnancies). Counterimmunoelectroforesis (CIE) and immunoblot (IB) were used to test antibodies to extractable nuclear antigens (ENA). Results: Only 2 cases of CHB (1.8%) were found among the 112 living newborns. In one case the mother with pri- mary Sjö gren' s Syndrome (pSS) was anti Ro 60 and 52kD positive while in the other case the mother affected by un- differentiated connective tissue disease (UCTD) was anti Ro 60kD and anti La positive. No fetal death was due to CHB. There were no cutaneous rashes at ...
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The aim of this study was to describe a single-center experience in the treatment and follow-up of cystoid macular edema patients. Clinical records of all patients with cystoid macular edema followed up in the Rheumatologic and... more
The aim of this study was to describe a single-center experience in the treatment and follow-up of cystoid macular edema patients. Clinical records of all patients with cystoid macular edema followed up in the Rheumatologic and Ophthalmological Unit of our center between 1993 and 2013 were retrospectively evaluated. The outcome was assessed by visual acuity and optical coherence tomography status during follow-up. Comparisons were made by Fisher&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s exact test (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.05 significant). In this study 16 eyes in 9 patients were analyzed. Our study includes mainly post-uveitic (78 %) cases with a high prevalence of human leukocyte antigen B51 (67 %). Systemic immunosuppressive therapy was prescribed in 87 % of cases. The most frequently used drugs were cyclosporine, interferon-α, and infliximab. The first two molecules appeared respectively the most used as the first option and the one with the longest survival on treatment. Interferon-α was the most effective drug in contrasting visual acuity loss compared to the majority of drugs, but significantly more effective than mycophenolate (p = 0.01) in reducing macular edema. At the end of follow-up, 50 % of patients showed a significant visual loss, while 88 % did not present macular edema. In our small cohort, interferon-α is the most promising drug in contrasting visual acuity loss in cystoid macular edema. Visual prognosis remains severe in these patients.
Research Interests: Treatment Outcome, Optical coherence tomography, Visual acuity, Humans, Female, and 15 moreMale, Follow-up studies, Cyclosporine, Clinical Sciences, Prevalence, Uveitis, Middle Aged, Adult, Retrospective Studies, Macular edema, Cohort Studies, Interferon Alpha, Immunosuppressive Agents, Infliximab, and Mycophenolic Acid
To assess the prevalence of disease- and therapy-related complications and of the organ damage after a follow-up of 15 years or more in patients with primary antiphospholipid syndrome (PAPS). Medical records of patients prospectively... more
To assess the prevalence of disease- and therapy-related complications and of the organ damage after a follow-up of 15 years or more in patients with primary antiphospholipid syndrome (PAPS). Medical records of patients prospectively followed in our centre for at least 15 years were retrospectively reviewed. Thirty-five Caucasian patients (33 female, two male) with diagnosis of PAPS followed from 1984 to 2013 with a mean age at onset of 32 years (SD 8.17) and a median follow-up of 20.5 years (range 15-30) were included. The occurrence of systemic autoimmune disease was observed in 14% of patients. Haemorrhagic, infective and neoplastic events were recorded in 34%, 6% and 9% respectively. Organ damage was present in 20% of patients at the end of the follow-up (17% neurological and 3% renal) and was significantly associated with the occurrence of thrombotic events (p: 0.027), particularly arterial (p&lt;0.001). A 48-year-old patient died from sepsis. During long-term follow-up of PAPS systemic autoimmunity is not unexpected. Organ damage progresses in a significant proportion of patients especially if they have suffered previous arterial events. Our study clearly shows the possible evolution of the disease and of organ damage, suggesting that optimal therapy and optimal prophylaxis of each PAPS patient should be carefully identified and strictly applied.
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SUMMARY Objective: to analyse efficacy and safety of anti-TNFα treatment in 17 patients with rheumatoid arthritis (AR) and an- ti-Ro antibodies, in order to detect difference in clinical and immunological response. Methods: 322 patients,... more
SUMMARY Objective: to analyse efficacy and safety of anti-TNFα treatment in 17 patients with rheumatoid arthritis (AR) and an- ti-Ro antibodies, in order to detect difference in clinical and immunological response. Methods: 322 patients, affected by RA and treated with anti-TNFα drugs, were considered, searching every 6-12 months ANA, anti-dsDNA and anti-ENA antibodies. Seventeen were anti-Ro positive and 305 anti-Ro negative before starting treatment. Results: anti-Ro positive subjects showed active arthritis at baseline (mean DAS: 5), with frequent extra-articular fea- tures, such as ocular and oral sicca symptoms. They showed rapid and stable improvement during the treatment, with- out significant difference compared to anti-Ro negative group. A good clinical Eular response was shown in 46% of anti-Ro negative subjects, steady stable during time. On the contrary, fewer anti-Ro positive patients seem to be "good" responders. RA remission (DAS <1,6) was achieved in 9-2...
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Patients with rheumatoid arthritis (RA) are at increased risk of vascular events. Data on the effects of tumor necrosis factor-α (TNF-α) blocking agents on aortic vascular function are still debated. To evaluate the effects of anti-TNF-α... more
Patients with rheumatoid arthritis (RA) are at increased risk of vascular events. Data on the effects of tumor necrosis factor-α (TNF-α) blocking agents on aortic vascular function are still debated. To evaluate the effects of anti-TNF-α treatment on elastic properties of the ascending aorta (distensibility, stiffness, and tissue Doppler imaging [TDI] strain) in RA patients. We prospectively followed 13 patients affected by RA without cardiovascular risk factors for 1 year during anti-TNF-α treatment. Every subject received an echocardiographic examination before starting anti-TNF-α drugs and after 1 year. Aortic elastic properties were calculated from the echocardiographically derived thoracic aortic diameters, and TDI strain was measured on the wall of the ascending aorta 3 cm above the aortic valve. We found lower distensibility (12.9 ± 3.5 vs 21.5 ± 7.5 mm Hg(-1); P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;0.001) and a higher stiffness index (21.3 ± 3.6 vs 11.7 ± 1.4; P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;0.001) in RA cases at baseline compared with values after 1 year of treatment. Peak systolic (S&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;) and diastolic (E&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39; and A&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;) waves of the aortic wall TDI were similar at baseline and at 1 year follow-up (S&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39; wave: 5.6 ± 2.2 cm/s vs 6.5 ± 2.6 cm/s, E&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39; wave: -4.6 ± 2.9 vs -5.0 ± 1.2 cm/s, A&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39; wave: -5.6 ± 0.19 vs -5.9 ± 2.05 cm/s), whereas TDI strain of the aortic wall was improved after anti-TNF-α treatment (-23.7 ± 1.4% vs -31.6 ± 2.8%, P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.001). Anti-TNF-α treatment after 12 months significantly modifies the elastic properties of the aorta. This may reflect the favorable changes in its elastic tissue after anti-TNF-α treatment in RA patients without cardiovascular risk factors. This suggests a potential cardiovascular risk benefit.
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Patients with rheumatoid arthritis (RA) are at increased risk of vascular events. Data on the effects of tumor necrosis factor-α (TNF-α) blocking agents on aortic vascular function are still debated. To evaluate the effects of anti-TNF-α... more
Patients with rheumatoid arthritis (RA) are at increased risk of vascular events. Data on the effects of tumor necrosis factor-α (TNF-α) blocking agents on aortic vascular function are still debated. To evaluate the effects of anti-TNF-α treatment on elastic properties of the ascending aorta (distensibility, stiffness, and tissue Doppler imaging [TDI] strain) in RA patients. We prospectively followed 13 patients affected by RA without cardiovascular risk factors for 1 year during anti-TNF-α treatment. Every subject received an echocardiographic examination before starting anti-TNF-α drugs and after 1 year. Aortic elastic properties were calculated from the echocardiographically derived thoracic aortic diameters, and TDI strain was measured on the wall of the ascending aorta 3 cm above the aortic valve. We found lower distensibility (12.9 ± 3.5 vs 21.5 ± 7.5 mm Hg(-1); P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;0.001) and a higher stiffness index (21.3 ± 3.6 vs 11.7 ± 1.4; P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;0.001) in RA cases at baseline compared with values after 1 year of treatment. Peak systolic (S&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;) and diastolic (E&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39; and A&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;) waves of the aortic wall TDI were similar at baseline and at 1 year follow-up (S&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39; wave: 5.6 ± 2.2 cm/s vs 6.5 ± 2.6 cm/s, E&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39; wave: -4.6 ± 2.9 vs -5.0 ± 1.2 cm/s, A&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39; wave: -5.6 ± 0.19 vs -5.9 ± 2.05 cm/s), whereas TDI strain of the aortic wall was improved after anti-TNF-α treatment (-23.7 ± 1.4% vs -31.6 ± 2.8%, P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.001). Anti-TNF-α treatment after 12 months significantly modifies the elastic properties of the aorta. This may reflect the favorable changes in its elastic tissue after anti-TNF-α treatment in RA patients without cardiovascular risk factors. This suggests a potential cardiovascular risk benefit.
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ABSTRACT
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Research Interests: Biopsy, Humans, Female, Male, Bone marrow, and 5 moreElectromyography, Clinical Sciences, Aged, Middle Aged, and Myositis
To assess the longterm cardiologic and immunologic outcome of children with isolated congenital complete heart block (CCHB) and their HLA antigens. Sixteen children with isolated CCHB were investigated. HLA typing was done using a... more
To assess the longterm cardiologic and immunologic outcome of children with isolated congenital complete heart block (CCHB) and their HLA antigens. Sixteen children with isolated CCHB were investigated. HLA typing was done using a microcytotoxicity test. Three patients died (18.7%), one in utero (35 weeks), one 2 days after birth, and one at 6 years of age. The mean age of the 13 living children is now 18.3 years (range 2-34). Eight (50%) have been permanently paced for symptoms. No patient developed clinical symptoms or serological abnormalities suggesting immune disease. The A31 antigen was more prevalent, but one pair of HLA identical twins was observed, and only one had CCHB. Patients with isolated CCHB have significant cardiac mortality, and after a long followup many of them are paced to control symptoms, but in our small sample those who survive the perinatal period mostly lead a normal life. The longterm immunological outcome of these children seems good. CCHB is not related...
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The Ro/La system is considered as an heterogeneous antigenic complex, constituted by three different proteins (52 kDa Ro, 60 kDa Ro and La) and four small RNAs particles. Anti-Ro/SSA are the most prevalent specificity among many... more
The Ro/La system is considered as an heterogeneous antigenic complex, constituted by three different proteins (52 kDa Ro, 60 kDa Ro and La) and four small RNAs particles. Anti-Ro/SSA are the most prevalent specificity among many autoimmune diseases, such as systemic lupus erythematosus (SLE), SS/SLE overlap syndrome, subacute cutaneous LE (SCLE), neonatal lupus and primary biliary cirrhosis. In contrast, anti-La/SSB is more associated with Sjögren&#39;s syndrome (SS). The differences between 52 kDa, 60 kDa Ro and La could explain why different assays did not show equivalent performance in anti-Ro and anti-La autoantibodies detection. The RNA precipitation assay had the highest sensitivity and specificity, usually considered as the reference methods. CIE is considered the most reliable to detect anti-Ro/SSA antibodies in routine practice, performing better than immunoblotting (IB) and some ELISAs. It shows a high sensitivity (89%) and specificity (100%). ELISA is generally considered a safe, rapid, sensitive and specific tecnique. Therefore, its high sensitivity often corresponds to a very low clinical specificity and the assay can give false positive results. Therefore, it is very important to search anti-Ro and anti-La only in selected patients, using the assay with high specificity and good predictive value, in order to have clinically significant and true positive results.
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Polymyositis/dermatomyositis (PM/DM) is an autoimmune disease characterised by skin and muscle inflammation, internal organ involvement and serum disease-specific autoantibodies. The recently identified anti-MDA5 (melanoma... more
Polymyositis/dermatomyositis (PM/DM) is an autoimmune disease characterised by skin and muscle inflammation, internal organ involvement and serum disease-specific autoantibodies. The recently identified anti-MDA5 (melanoma differentiation-associated gene 5) antibodies are associated with clinically amyopathic DM (CADM), rapidly progressive interstitial lung disease, severe skin manifestations, and poor prognosis. Our objective was to examine the clinical significance of anti-MDA5 antibodies in a cohort of European Caucasian patients with PM/DM, considering that data on anti-MDA5 serology are limited to Asian and US cohorts. Sera from 76 consecutive adult Italian patients with PM/DM were analysed by immunoprecipitation (IP) of 35S-methionine radiolabelled HeLa and K562 cell extracts, ELISA using recombinant MDA5 protein and IP-Western Blot using rabbit anti-MDA5 antibodies. Clinical associations of anti-MDA5 antibody positive patients were analysed. Anti-MDA5 antibodies were identifi...
Research Interests: Western blotting, Italy, Humans, Female, Male, and 15 morePhenotype, Immunoprecipitation, Clinical Sciences, Aged, Prevalence, Middle Aged, Adult, HeLa cells, Retrospective Studies, European Continental Ancestry Group, Biological markers, Dermatomyositis, Enzyme Linked Immunosorbent Assay, Predictive value of tests, and Autoantibodies
To assess the longterm cardiologic and immunologic outcome of children with isolated congenital complete heart block (CCHB) and their HLA antigens. Sixteen children with isolated CCHB were investigated. HLA typing was done using a... more
To assess the longterm cardiologic and immunologic outcome of children with isolated congenital complete heart block (CCHB) and their HLA antigens. Sixteen children with isolated CCHB were investigated. HLA typing was done using a microcytotoxicity test. Three patients died (18.7%), one in utero (35 weeks), one 2 days after birth, and one at 6 years of age. The mean age of the 13 living children is now 18.3 years (range 2-34). Eight (50%) have been permanently paced for symptoms. No patient developed clinical symptoms or serological abnormalities suggesting immune disease. The A31 antigen was more prevalent, but one pair of HLA identical twins was observed, and only one had CCHB. Patients with isolated CCHB have significant cardiac mortality, and after a long followup many of them are paced to control symptoms, but in our small sample those who survive the perinatal period mostly lead a normal life. The longterm immunological outcome of these children seems good. CCHB is not related to a specific HLA pattern in affected children.
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Lymphocyte subsets were analyzed in peripheral blood and lymph nodes from a drug-addict with acquired immunodeficiency syndrome (AIDS) presenting with disseminated lymphadenopathic and oropharyngeal Kaposi&#39;s sarcoma. At the onset... more
Lymphocyte subsets were analyzed in peripheral blood and lymph nodes from a drug-addict with acquired immunodeficiency syndrome (AIDS) presenting with disseminated lymphadenopathic and oropharyngeal Kaposi&#39;s sarcoma. At the onset of disease, hypergammaglobulinemia, increase of OKT8+ T cell subset and reversal of OKT4/OKT8 ratio were found in the blood. At the same time, lymph nodes displayed, besides Kaposi&#39;s sarcoma, marked follicular hyperplasia, plasmocytosis and increase of OKT8+/Leu 2a+ T cells within follicular centers. These results are interpreted to indicate that at an early stage of disease the major tissue alterations took place within follicular centers and consisted of both B cell activation and T suppressor cell reaction. These changes correlated with immunological abnormalities observed in peripheral blood. Immunohistochemical investigation of lymphoid tissue may be useful to detect AIDS patients at an early stage.
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Serum prealbumin is a recognized marker of malnutrition, but its role in the prognosis of patients with SSc has not yet been investigated. The aim of the present multicentre prospective study was to investigate the association between... more
Serum prealbumin is a recognized marker of malnutrition, but its role in the prognosis of patients with SSc has not yet been investigated. The aim of the present multicentre prospective study was to investigate the association between prealbumin and mortality, independent of clinical features, in a cohort of SSc outpatients. Patients were followed up according to standard clinical guidelines with visits at least every 6 months. Data collected included records of skin and internal organ involvement, survival and causes of death. During a median follow-up of 48 months [interquartile range (IQR) 25-58], 34/299 patients (11%) died. In univariable survival analysis, age; male sex; lung, gastrointestinal or multiple visceral organ involvement (two or more); co-morbidities (two or more) and low serum prealbumin were significant predictors of mortality. In bivariable Cox models, alternatively adjusted for significant predictors, prealbumin was independently and significantly associated with...
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Studies suggest that anti-52 kDa Ro antibodies are more sensitive and specific than anti-60 kDa Ro antibodies for neonatal lupus. However, these studies mainly used immunoblot or ELISA using recombinant protein, which have poor... more
Studies suggest that anti-52 kDa Ro antibodies are more sensitive and specific than anti-60 kDa Ro antibodies for neonatal lupus. However, these studies mainly used immunoblot or ELISA using recombinant protein, which have poor sensitivity for anti-60 kDa Ro antibodies. In addition, the control patients were not disease matched. We reassessed the sensitivity and specificity of anti-52 kDa Ro, anti-60 kDa Ro, and anti-La, addressing these limitations. To assess sensitivity, 125 mothers of children with neonatal lupus (NLM) were recruited. All maternal sera were assessed using a commercial line immunoassay that uses natural 60 kDa Ro protein (Inno-Lia ANA Update, Innogenetics NV, Gent, Belgium). By this method, 96% of the sera had antibodies to 60 kDa Ro, 86% to 52 kDa Ro, and 78% to 48 kDa La. Immunoblot of 65 NLM showed significantly fewer positive results for anti-60 kDa Ro (p < 0.001) and anti-52 kDa Ro (p < 0.05). Sensitivity of the 3 antibodies was assessed in the symptoma...
Research Interests: Immunology, Multivariate Analysis, Humans, Systemic Lupus Erythematosus, Female, and 15 moreMale, Infant, Follow-up studies, The, Clinical Sciences, Newborn Infant, Public health systems and services research, Biological markers, Disease Progression, Sensitivity and Specificity, Reference Values, Enzyme Linked Immunosorbent Assay, Logistic Models, Autoantibodies, and Cohort Studies
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Research Interests: Immunology, Autoimmunity, Adolescent, Antibodies, Humans, and 15 moreSystemic Lupus Erythematosus, Female, Rheumatic Heart Disease, Male, Ribosomal proteins, Inflammatory disease, Aged, Middle Aged, Adult, Diagnostic Test, Protozoan Proteins, Sensitivity and Specificity, Healthy Subjects, Enzyme Linked Immunosorbent Assay, and Immunoblotting
Research Interests: Echocardiography, Elasticity, Humans, Female, Male, and 4 moreHeart, Middle Aged, Aorta, and Case Control Studies
Research Interests: Mental Health, Depression, Quality of life, Rheumatoid Arthritis, Translations, and 19 moreHumans, Health related Quality of Life, Systemic sclerosis, Female, Clinical, Male, Disease Severity, STEM (short form), Beck Depression Inventory, Clinical Sciences, General Health, Aged, Middle Aged, Questionnaires, Prognosis, Reproducibility of Results, Depressive Symptoms, Indexation, and Disease Activity
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Research Interests: Demography, Immunology, Language, Italy, Humans, and 16 moreFemale, Male, Central Nervous System, Arthritis, Health Status, Clinical Sciences, Aged, Middle Aged, Adult, Public health systems and services research, Sjogren´s Syndrome, Disease Activity, Xerophthalmia, Cohort Studies, Salivary Glands, and Motor activity
Research Interests: Immunology, Electrocardiography, Prospective studies, Humans, Systemic Lupus Erythematosus, and 14 moreFemale, Male, Arthritis, Risk factors, Clinical Sciences, Newborn Infant, Prevalence, Public health systems and services research, Biological markers, Risk Factors, Ribonucleoproteins, Prospective Study, Autoantigens, and Bradycardia
To analyze the performance of a line blot assay for the identification of autoantibodies in sera of patients affected by myositis, compared with immunoprecipitation (IP) as gold standard. 66 sera of patients with myositis (23... more
To analyze the performance of a line blot assay for the identification of autoantibodies in sera of patients affected by myositis, compared with immunoprecipitation (IP) as gold standard. 66 sera of patients with myositis (23 polymyositis, 8 anti-synthetase syndromes, 29 dermatomyositis and 6 overlap syndromes) were tested by commercial LB (Euroimmun, Lubeck, Germany); 57 sera were analyzed also by IP of K562 cell extract radiolabeled with (35)S-methionine. Inter-rater agreement was calculated with Cohen&amp;amp;amp;amp;#39;s k coefficient. Myositis-specific antibodies (MSA) were detected in 36/57 sera (63%) by IP and in 39/66 sera (59%) by LB. The most frequent MSA found by LB were anti-Jo1 and anti-Mi2 found in 15% (10/66) of sera, followed by anti-NXP2 and anti-SRP detected in 106% (7/66) of sera. Anti-TIF1gamma and anti-MDA5 were found in 6 (9%) and 5 sera (7.6%), respectively. A good agreement between methods was found only for anti-TIF1γ, anti-MDA5 and anti-NXP-2 antibodies, while a moderate agreement was estimated for anti-Mi2 and anti-EJ. By contrast, a high discordance rate for the detection of anti-Jo1 antibodies was evident (k: 0.3). Multiple positivity for MSA were found in 11/66 (17%) by LB and 0/57 by IP (p: 0001). Comparing the clinical features of these 11 sera, we found total discrepancies between assays in 3 sera (27.3%), a relative discrepancy due to the occurrence of one discordant autoantibody (not confirmed by IP) in 5 cases (45.5%) and a total discrepancy between LB and IP results, but with a relative concordance with clinical features were found in other 3 sera (27.3%). The semiquantitative results do not support the interpretation of the data. The use of LB assay allowed the detection of new MSA, such as anti-MDA5, anti-MJ and anti-TIF1gamma antibodies, previously not found with routine methods. However, the high prevalence of multiple positivities and the high discondant rate of anti-Jo1 antibodies could create some misinterpretation of the results from the clinical point of view. These data should be confirmed by enlarging the number of myositis cases.
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Idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of myositis, characterised by chronic muscle weakness, cutaneous features, different extra-muscular manifestations and circulating autoantibodies. IIMs included classical... more
Idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of myositis, characterised by chronic muscle weakness, cutaneous features, different extra-muscular manifestations and circulating autoantibodies. IIMs included classical polymyositis (PM), dermatomyositis (DM) and other different types of myositis with a wide range of muscle involvement. A complete autoantibody profile and a muscle biopsy are mandatory to correctly diagnose different clinical entities and to define their different prognosis. Bohan and Peter&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s criteria included five items to diagnose adult onset PM and DM. The sensitivity was 74-100 %, while the specificity is low, due to a poor ability to differentiate PM from neuromuscular diseases. Other criteria included a more accurate histological definition of PM, DM or amyopathic DM, obtaining a higher specificity. Autoantibodies&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39; association, interstitial lung disease and clinical cardiac involvement represent the main items that could define the prognosis of these patients. On the other hand, inclusion body myositis is a different myopathy characterised by a peculiar muscle mass involvement, muscle atrophy and progressive loss of function, due to complete failure to all immunosuppressive drugs used. Treatment of IIMs is based on corticosteroids (CS), which show rapid clinical response and functional improvement. Different immunosuppressant drugs are given to obtain a better control of the disease during CS tapering dose. No controlled double blind trials demonstrated the superiority of one immunesuppressant on another. The occurrence of interstitial lung involvement requires the immediate introduction of immunosuppressants in addiction to CS. Severe dysphagia seems to improve with intravenous immunoglobulins (Ig). Physical therapy could be started after the acute phase of diseases and seems to have a beneficial role in muscle strength recovery.
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Research Interests: Adolescent, Spleen, Humans, Child, Female, and 6 moreMale, Middle Aged, Adult, Lymphocytes, Immunoglobulins, and Splenectomy
Lymphocytosis of large granular lymphocytes (LGL) has been observed in 6 patients splenectomized for various pathological conditions. In all of them the LGL count was higher than 3.5 x 10(9)/l. No patient showed neutropenia nor suffered... more
Lymphocytosis of large granular lymphocytes (LGL) has been observed in 6 patients splenectomized for various pathological conditions. In all of them the LGL count was higher than 3.5 x 10(9)/l. No patient showed neutropenia nor suffered from rheumatoid arthritis. A surface markers heterogeneity was observed by immunophenotypic studies. A reversal of the CD4/CD8 ratio was observed in all patients, indicating that LGL are in the majority CD8+. Three patients showed the phenotype CD2+ CD3+ CD4- CD8+ indicating the T-lineage derivation of LGL; patient 6 showed a non-T non-B phenotype (CD2- CD3- CD4- CD8+/-). The percentage of lymphocytes presenting LGL-related markers (HNK-1, CD16, CD11b) was higher than that observed in normal subjects in 4 out of 5 examined patients. However, the percentage of cells bearing these markers was inferior to the LGL counts indicating that not all LGL express them. NK cytotoxic activity was similar to that of normal subjects in the three examined patients. Our data suggest that lymphocytosis of LGL in splenectomized subjects is a reactive process favoured by the asplenic state.
Research Interests: Humans, Female, Male, Aged, Middle Aged, and 4 moreAdult, Lymphocytes, Lymphocytosis, and Splenectomy
Kaposi&#39;s Sarcoma (KS) in young individuals is unusual and most often associated with cellular immunodeficiency caused by infective or other neoplastic diseases. It has recently been highly associated with the Acquired... more
Kaposi&#39;s Sarcoma (KS) in young individuals is unusual and most often associated with cellular immunodeficiency caused by infective or other neoplastic diseases. It has recently been highly associated with the Acquired Immunodeficiency Syndrome (AIDS). We report the case of a heterosexual 29 year aged man with no evidence of underlying malignancy or infectious diseases. Antibodies to the Human Immunodeficiency Virus (HIV) were absent on repeat testing. His immunological profile demonstrated elevated number of CD8+ cells, normal number of CD3+ and CD4+ cells and hypogammaglobulinemia. These data are distinctly different from those described with AIDS associated KS. The development of KS in young individuals of mediterranean origin may reflect mild degree of immune abnormalities in the absence of infection with HIV.
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Research Interests: Medicine, Humans, Female, Male, Arthritis, and 6 moreClinical Sciences, Aged, Middle Aged, Adult, Myositis, and Retrospective Studies
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Subcutaneous (SC) abatacept (ABA) is comparable to intravenous (IV) formulation in terms of efficacy and safety profile. Our work analyzed the switch to SC formulation from IV administration in patients with rheumatoid arthritis.... more
Subcutaneous (SC) abatacept (ABA) is comparable to intravenous (IV) formulation in terms of efficacy and safety profile. Our work analyzed the switch to SC formulation from IV administration in patients with rheumatoid arthritis. Fifty-one patients treated with SC ABA were included. Clinical data were obtained from clinical charts. Fourteen patients relapsed and needed to return to the IV administration. Neither clinical and laboratory features nor the previous therapies were identified as risk factors for SC formulation inefficacy. Disease activity decreased after the return to IV infusions. SC ABA showed a risk of relapse in 27% of cases. The reinsertion of the IV administration quickly reinstated disease control.
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Cardiac resynchronization therapy (CRT) is an effective therapy for patients with reduced systolic function and enlarged QRS. Recently, some Authors have demonstrated that the presence of positive antinuclear antibodies (ANAs) may play a... more
Cardiac resynchronization therapy (CRT) is an effective therapy for patients with reduced systolic function and enlarged QRS. Recently, some Authors have demonstrated that the presence of positive antinuclear antibodies (ANAs) may play a role in the development of heart failure in a population of patients implanted with PM. We investigated the effect of positive ANAs in 90 patients (mean age 71 ± 8 yrs) implanted with a CRT device in our Centre between May 2010 and June 2013. To assess for immunologic contribution to CRT outcome, patients were divided into positive and negative ANAs (ANA +, ANA ---), considering as positive patients with an ANAs dilution > 1:80. The primary end - point was constituted by a combined end - point of death or first hospitalization for heart failure; secondary end - points were constituted by: 1. incidence of first hospitalization for heart failure and 2.total cause mortality. After a mean follow - up of 1200 days, primary end - point occurred in 11 p...
Aseptic meningitis is a rare and aggressive complication of rheumatoid arthritis (RA), usually histologically characterised by rheumatoid nodules and lymphocytic aggregates in leptomeninges. The aim of this study was to describe the... more
Aseptic meningitis is a rare and aggressive complication of rheumatoid arthritis (RA), usually histologically characterised by rheumatoid nodules and lymphocytic aggregates in leptomeninges. The aim of this study was to describe the clinical onset and evolution of aseptic meningitis occurring during anti-TNF-alpha (TNF-α) therapy. we retrospectively analysed the clinical records of patients with RA or ankylosing apondylitis (AS) treated by TNF-α drugs in the last 10 years. Four out of 718 patients, treated with TNF-α, developed meningitis after a mean of 5 years (SD: 3.7) of TNF-α exposure (0.55%). Three subjects were affected by long-standing RA (median: 11 years, IQR:8.5-25), one patient by active AS of 8 years' duration. RA patients were treated with etanercept (2 cases) and infliximab (1 case), in association with methotrexate and prednisone. The AS patient was treated with adalimumab. Neurological onset was focal epilepsy (3 cases) and dysarthria (1 case). RM showed leptome...
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We aimed to analyse the annual incidence of anti-Ku antibodies and to study their clinical associations in patients mainly affected by systemic sclerosis (SSc) and systemic lupus erythematosus (SLE) overlap diseases. Anti-Ku were detected... more
We aimed to analyse the annual incidence of anti-Ku antibodies and to study their clinical associations in patients mainly affected by systemic sclerosis (SSc) and systemic lupus erythematosus (SLE) overlap diseases. Anti-Ku were detected by counterimmunoelectrophoresis in a total of 203 sera during 14 years of anti-ENA detection. Anti-Ku+ sera belong to 46 patients, mostly affected by UCTD (12 cases), SSc spectrum diseases (13 cases), including SSc/PM, SSc/DM and SSc; and SLE spectrum diseases (9 cases), including SLE, SLE/APS, SLE/SS, SLE/PM. Anti-Ku antibodies represent 2% of all anti-ENA positive sera, and are found in 1.3-3% of anti-ENA positive sera per year. Anti-Ku+ SSc represents 2% of all SSc patients. All anti-Ku+ SSc spectrum diseases showed a limited cutaneous disease; myositis, dysphagia and isolated anti-Ku in more than 70% of cases. Interstitial lung disease (ILD) was found in 54% of SSc patients, frequently with mild functional impairment. When compared with other l...
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To correlate the clinical course of the disease with the titer, the isotype profile and the switch of the anti-Ro/SSA antibodies in a cohort of patients affected by UCTD. One hundred selected patients with anti-Ro/SSA antibodies detected... more
To correlate the clinical course of the disease with the titer, the isotype profile and the switch of the anti-Ro/SSA antibodies in a cohort of patients affected by UCTD. One hundred selected patients with anti-Ro/SSA antibodies detected by counterimmunoelectrophoresis (CIE), and affected by UCTD with a mean follow-up of 7.6 years (SD 4.8 yrs.), were studied. The titer of IgA, IgG and IgM anti-Ro/SSA antibodies was determined in two different sera, obtained at the time of diagnosis and at the last visit, by ELISA with Ro/SSA recombinant proteins as substrate. Thirty-five patients evolved from UCTD to a different connective tissue disease, while 65 showed a stable disease. Anti-Ro/SSA antibodies were detected in 91% and 97% of the patients, at baseline and during follow-up, respectively. IgG dominates the anti-Ro response. The titer of IgA, IgM and IgG anti-Ro/SSA did not differ significantly between the two groups of patients with UCTD. An increasing trend of IgG and IgA anti-Ro/SSA...
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To assess the prevalence of anti-Ro/SSA in RA and to analyse clinical and serological features of anti-Ro/SSA positive patients with RA. 195 consecutive patients affected by RA were studied by counterimmunoelectrophoresis and ELISA for... more
To assess the prevalence of anti-Ro/SSA in RA and to analyse clinical and serological features of anti-Ro/SSA positive patients with RA. 195 consecutive patients affected by RA were studied by counterimmunoelectrophoresis and ELISA for the detection of anti-Ro/SSA antibodies. Anti-Ro were found in 12 patients, with a prevalence of 6%. These 12 patients were pooled with other 15 patients known to have anti-Ro/SSA antibodies and RA, in order to evaluate their clinical and laboratory features. Anti-Ro positive patients showed a common pattern of joint involvement at onset and a comparable progression of disease compared to anti-Ro negative subjects. In addition, extra-articular manifestations (such as xerophthalmia, xerostomia, scleritis, oral ulcers and amyloidosis) and peculiar autoantibody profile (hypergammaglobulinemia, anti-dsDNA and AMA) were found significantly associated to anti-Ro/SSA positivity. Even though DMARDs withdrawals were more frequently detected in anti-Ro/SSA pati...
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OBJECTIVE: To assess the prevalence of Congenital Heart Block (CHB) in newborns from anti Ro/SS-A antibodies positive mothers affected by connective tissue diseases (CTD) and to evaluate the prevalence of other manifestations of Neonatal... more
OBJECTIVE: To assess the prevalence of Congenital Heart Block (CHB) in newborns from anti Ro/SS-A antibodies positive mothers affected by connective tissue diseases (CTD) and to evaluate the prevalence of other manifestations of Neonatal Lupus (NL) and the electrocardiographic abnormalities. METHODS: A prospective study was conducted on 100 anti Ro/SS-A positive mothers that were followed before and during their 118 pregnancies (4 twin pregnancies and 18 second pregnancies). Counterimmunoelectroforesis (CIE) and immunoblot (IB) were used to test antibodies to extractable nuclear antigens (ENA). RESULTS: Only 2 cases of CHB (1.8%) were found among the 112 living newborns. In one case the mother with primary Sjögren's Syndrome (pSS) was anti Ro 60 and 52kD positive while in the other case the mother affected by undifferentiated connective tissue disease (UCTD) was anti Ro 60kD and anti La positive. No fetal death was due to CHB. There were no cutaneous rashes at birth while mild h...
To evaluate the clinical and serologic profile, the rate of progression to well defined CTD and the possible predictors of disease evolution in patients affected by UCTD with antibodies anti-RoISSA. 148 patients diagnosed as UCTD were... more
To evaluate the clinical and serologic profile, the rate of progression to well defined CTD and the possible predictors of disease evolution in patients affected by UCTD with antibodies anti-RoISSA. 148 patients diagnosed as UCTD were retrospectively evaluated. Antibodies to SSA/Ro were determined by counter-immunoelectrophoresis and ELISA. Thirty-six patients (24.3%) developed a well-defined CTD after a mean follow-up of 4.5 years. Most patients developed primary Sjögren's syndrome (SS) (50%) or systemic lupus erythematosus (SLE) (30.5%). Leukopenia and xerophthalmia developed more frequently in the group of patients evolving to defined CTDs (p < 0.0032 and p < 0.0063). Leukopenia independently predicted the evolution in CTD by multivariate regression analysis (p < 0.019). Anti-dsDNA predicted the evolution in SLE (p < 0.0207), while the presence of additional anti-ENA specificity to anti-Ro/SSA was not associated with the outcome. 24.3% of patients with UCTD and an...
Research Interests: Adolescent, Humans, Child, Systemic Lupus Erythematosus, Female, and 15 moreMale, Follow-up studies, Clinical Sciences, Aged, Middle Aged, Adult, Sjogren´s Syndrome, Retrospective Studies, Disease Progression, Mixed connective tissue disease, Ribonucleoproteins, Xerophthalmia, Enzyme Linked Immunosorbent Assay, Autoantigens, and Interleukin
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We evaluated 95 HIV seropositive drug-addicts during a follow-up period of one year. The patients were classified and reclassified at each visit, according to the criteria proposed by the Centers for Disease Control (CDC) in 1986. At the... more
We evaluated 95 HIV seropositive drug-addicts during a follow-up period of one year. The patients were classified and reclassified at each visit, according to the criteria proposed by the Centers for Disease Control (CDC) in 1986. At the first visit the patients were classified as follows: 70 in IIa-IIIa, 23 in IIb-IIIb and 2 in group IV. a and b indicate the absence or the presence of immunological and/or hematological alterations. 1 out of 70 IIa-IIIa patients versus 8 out of 23 IIb-IIIb patients developed AIDS in one year (p less than 0.001). Only one patient belonging to IIa-IIIa groups at the first visit progressed to AIDS in one year. This patient was reclassified in subgroup b after six months. No differences were noted among patients classified in IIb (5 patients) and IIIb (4 patients) who progressed to AIDS. Taken together these data indicate that the belonging to b subgroup is a risk factor for developing AIDS and the passage through a b subgroup (II or III) is a necessary...
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Flow cytometric 2-color analysis of peripheral blood lymphocytes from patients with systemic lupus erythematosus (SLE) showed a reduction of relative and absolute number of CD4+ CD29+ cells compared to matched healthy individuals. This... more
Flow cytometric 2-color analysis of peripheral blood lymphocytes from patients with systemic lupus erythematosus (SLE) showed a reduction of relative and absolute number of CD4+ CD29+ cells compared to matched healthy individuals. This abnormality was more marked in patients with active/very active disease. Absolute number of CD4+ CD29+ cells was negatively correlated with spontaneous anti-DNA Ig production that we demonstrated to be a laboratory index strongly correlated with a clinical disease activity score. A decrease of the percentage of CD8+ CD29+ lymphocytes in patients with active disease was also observed.
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Patients with systemic lupus erythematosus (SLE) are prone to hypo-vitaminosis D because of their photosensitivity. Vitamin D (vit.D) has beneficial effects not only on bone metabolism but also on the function of the immune system. The... more
Patients with systemic lupus erythematosus (SLE) are prone to hypo-vitaminosis D because of their photosensitivity. Vitamin D (vit.D) has beneficial effects not only on bone metabolism but also on the function of the immune system. The relationship between SLE disease activity and vit.D status is controversial and little is known on the effects of current supplementation strategies given for osteoporosis in raising vit.D levels. Vit.D levels were measured longitudinally in 50 SLE patients from Northern Italy at two time-points (winter and summer) during disease remission. Thirty patients were also evaluated during a flare. As controls, 170 healthy donors were enrolled. All the samples were analysed for 25-OH vit.D levels by a chemiluminescence assay (DiaSorin SpA, Italy). SLE patients had lower vit.D levels than controls in the summer (median 29.4 vs. 39.2 ng/ml, p=0.005) but not in the winter (26.4 vs. 21.6 ng/ml). During wintertime, 36 SLE patients were supplemented with vit.D dro...
Research Interests: Vitamin D, Calcium, Treatment Outcome, Adolescent, Italy, and 20 moreHumans, Systemic Lupus Erythematosus, Female, Male, Young Adult, Dietary Supplements, Risk factors, Recurrence, Clinical Sciences, Aged, Middle Aged, Longitudinal Studies, Adult, Time Factors, Retrospective Studies, Tablets, Biological markers, Risk Factors, Seasons, and Vitamin D Deficiency
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IgA antiphospholipid antibodies (aPL) are not currently recognized as formal laboratory criteria for the Antiphospholipid Syndrome (APS). This is mainly due to methodological issues (different study designs, use of various... more
IgA antiphospholipid antibodies (aPL) are not currently recognized as formal laboratory criteria for the Antiphospholipid Syndrome (APS). This is mainly due to methodological issues (different study designs, use of various non-standardized IgA assays). However, there are experimental data showing the pathogenic role of IgA anti-cardiolipin antibodies (aCL) and IgA anti-β2glycoprotein I antibodies (anti-β2GPI). Isolated IgA aCL are not very common, therefore their testing could be useful in the case of strong suspicion of APS but negative results for other aPL tests. IgA anti-β2GPI seem to be the most prevalent isotype in patients with Systemic Lupus Erythematosus (SLE), with a significant association with thrombotic events. Such a clinical relevance has been recently recognized by the inclusion of these autoantibodies among the aPL tests in the novel SLICC classification criteria for SLE. Emerging interest has been raised by IgA anti-β2GPI against domain 4/5 as a novel subgroup of clinically relevant aPL.
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ABSTRACT Background Systemic lupus erythematosus (SLE) predominantly affects young women in the third decade of life. A small subset of patients develops SLE after 50 years of age. There is controversy whether older-onset SLE is... more
ABSTRACT Background Systemic lupus erythematosus (SLE) predominantly affects young women in the third decade of life. A small subset of patients develops SLE after 50 years of age. There is controversy whether older-onset SLE is associated with a more benign disease course than younger-onset SLE. Objectives To compare disease activity, clinical and immunological features as well as the rate and cause of mortality between 535 SLE patients with early or late onset, diagnosed according to ACR criteria revised in 1997, attending our outpatients’ clinic from 1976 to 2008. Methods Patients were divided in two groups according to the onset of SLE before or after 50 years of age. Forty patients (7.5%) presented a late onset (Group A), while 495 (92.5%) (Group B) showed an early onset of SLE. Clinical data (musculo-skeletal manifestations, mucocutaneous symptoms, constitutional symptoms, serositis, mayor organ involvement and symptoms related to antiphospholipid antibodies syndrome), laboratory and immunological assessment (C3, C4, CH50, rheumatoid factor, antinuclear anti-dsDNA antibodies, anti-ENA, anticardiolipin and anti-beta 2 glycoprotein I antibodies, Lupus anticoagulant) were derived from the clinical records. Disease activity was measured by ECLAM score (1) at the time of SLE diagnosis. The two groups were compared by Student’s t test, Chi square or Fisher’s exact tests when required. Results Comparing the clinical features of SLE, sicca symptoms were significantly more frequent in group A (p&lt;0.015), while glomerulonephritis was prevalent in early onset SLE (p&lt;0.016). Analyzing the immunological features, we found that C3 was more frequently reduced in group B (p&lt;0.0005) as well as ANA were more frequently detected in group B (p&lt;0.02). No difference was detected for anti-dsDNA, anti-phospholipid or anti-ENA antibodies’ distribution between groups.The mean of ECLAM score was 3.69 (±1.5) in Group A versus 4.11 (±2.3) in Group B. A total of 22 patients (4.25%) died during follow-up: 14 in group B (2.8%) and 8 in group A (20%). Duration of follow -up in the two groups was not significantly different even if group B was followed for 14.9 years (SD: 9.5) and group A for 11 years (SD: 6.3). ECLAM scored 4.12 (±1.09) in Group A versus 5.03 (±2.3) in Goup B of patient who died during follow-up. The most frequent cause of death was represented by infections in group B (28.5%) and cardiovascular (CV) events in group A (50%). Myocardial infarction and stroke rates did not differ in the two groups (21.5% in group B vs 25% in group A) and they are invariably associated to anti-phospholipid antibodies. Conclusions Late onset SLE occurred in 7,5% of patients and differed from early onset SLE for a more frequent occurrence of sicca and lower prevalence of nephritis, C3 reduction and ANA positivity. Deaths occurred more frequently in late onset SLE mainly for CV involvement. Disease activity as measured by ECLAM was slightly higher in Group B versus Group A while not significantly different. Disclosure of Interest None Declared
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... editors Immunological Abnormalities in the Antiphospholipid Syndrome Angela Tincani1, Franco Franceschini1, Michela Spunghi1, Paola Panzeri2, Genesio Balestrieri1 and Pier Luigi Meroni2 &#x27;Servizio di Reumatologia, Allergologia... more
... editors Immunological Abnormalities in the Antiphospholipid Syndrome Angela Tincani1, Franco Franceschini1, Michela Spunghi1, Paola Panzeri2, Genesio Balestrieri1 and Pier Luigi Meroni2 &#x27;Servizio di Reumatologia, Allergologia e Immunologia Clinica, Spedali Civili ...
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The objective of this study was to analyse clinical and serological associations of anti-Ki antibodies. Thirty-five patients with anti-Ki antibodies, detected by CIE, selected from laboratory routine, were studied. All patients were... more
The objective of this study was to analyse clinical and serological associations of anti-Ki antibodies. Thirty-five patients with anti-Ki antibodies, detected by CIE, selected from laboratory routine, were studied. All patients were affected by autoimmune diseases: SLE and pSS were the most frequent diagnoses. The cohort was constituted by 27 female and eight males. Main clinical features were skin involvement (60%), xerophtalmia (48.6%), Raynaud&#39;s phenomenon (43%), photosensitivity (34%), xerostomia (31.4%). CNS involvement was present in four (11.4%) and renal disease in seven cases (20%). ANA, anti-dsDNA and RF were detected in 100%, 60% and 34.5%. In SLE, anti-Ki was detected in 6% of cases, more frequently in males compared to other SLE patients without anti-Ki (P &lt; 0.004). Nineteen anti-Ki positive patients affected by SLE showed more frequently malar rash and multiple autoantibody specificities compared to 16 anti-Ki positive patients with other diseases (P = 0.044 and P = 0.0003, respectively). Our study confirms a preferential occurrence of anti-Ki antibodies in patients with sicca and skin involvement. Malar rash and multiple ANA specificities were significantly associated with SLE compared to other diseases in our study. Anti-Ki were detected in 6% of patients with SLE with a significant prevalence in males.
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Thirteen patients with systemic lupus erythematosus and deforming arthropathy (DA) of the hands were compared with 111 patients with SLE without deforming arthropathy. Clinical features were comparable in the two groups. Patients... more
Thirteen patients with systemic lupus erythematosus and deforming arthropathy (DA) of the hands were compared with 111 patients with SLE without deforming arthropathy. Clinical features were comparable in the two groups. Patients fulfilling criteria for mixed connective tissue disease (MCTD) were not included in the present study. A higher prevalence of antibodies to SSA/Ro (P &lt; 0.0125) and SSB/La (P &lt; 0.004) were found in the SLE-DA group. The detection of antibodies to SSA/Ro and SSB/La was even more strictly associated with DA in SLE antibodies to SSA/Ro alone (P &lt; 0.002). Regarding the fine specificity of anti-SSA/Ro, a prevalent response to the 52 kD protein of the Ro antigen was found. We conclude that patients with SLE developing deformities of the hands belong to a subset of patients with circulating antibodies to SSA/Ro, particularly to the 52 kD component, and to SSB/La.
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Previous investigations of antinuclear antibody (ANA) prevalence in patients undergoing PUVA therapy reported contrasting results. However, ANA tests were performed on low-sensitivity substrates that do not allow investigation of anti-Ro... more
Previous investigations of antinuclear antibody (ANA) prevalence in patients undergoing PUVA therapy reported contrasting results. However, ANA tests were performed on low-sensitivity substrates that do not allow investigation of anti-Ro (SS-A) antibodies. We assessed ANAs on a highly sensitive substrate. ANAs were assayed on HEp-2 cells at regular intervals in 238 patients with psoriasis who were treated with PUVA therapy for 1 to 5 years and in 118 untreated control subjects with psoriasis. In addition, radioimmunoassay and counterimmunoelectrophoresis studies of anti-DNA and anti-extractable nuclear antigen antibodies were performed. Low titers of ANA developed in three patients in at least two consecutive determinations and in 10 patients in a single determination despite continuing treatments. The positive conversion rate was not statistically significant. Radioimmunoassay counterimmunoelectrophoresis studies of anti-DNA and anti-extractable nuclear antigen antibodies were never positive. In our experience PUVA therapy does not represent a risk factor for the induction of anti-Ro antibodies and other ANAs.
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Three patients are described with clinical features of connective tissue diseases, namely, dermatomyositis, progressive systemic scleroderma, and systemic lupus erythematosus. In two patients the symptoms of disease overlapped. The rare... more
Three patients are described with clinical features of connective tissue diseases, namely, dermatomyositis, progressive systemic scleroderma, and systemic lupus erythematosus. In two patients the symptoms of disease overlapped. The rare anti-Ku antibody was found in all of them. Anti-Ku antibody characterizes those patients, with polymyositis-scleroderma overlap syndromes who have a good prognosis. One of our patients, who also had severe anti-Sm-positive systemic lupus erythematosus, seems to be an exception.
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Patients with scleroderma (systemic sclerosis; SSc) can be classified into subsets based on autoantibody profile and clinical features. Specificities such as anti-Th/To and anti-fibrillarin (U3RNP) are detectable mainly by... more
Patients with scleroderma (systemic sclerosis; SSc) can be classified into subsets based on autoantibody profile and clinical features. Specificities such as anti-Th/To and anti-fibrillarin (U3RNP) are detectable mainly by immunoprecipitation (IP), which is not widely used in clinical laboratories. We examined the autoantibody profiles and clinical manifestations in a cohort of Italian patients with SSc, focusing on anti-Th/To and anticentromere (ACA) antibodies, associated with limited cutaneous SSc (lcSSc). Sera from 216 consecutive patients with SSc were tested for ACA (by indirect immunofluorescence), antitopoisomerase I (topo I; by counterimmunoelectrophoresis), and anti-RNA polymerase III (RNAPIII; by ELISA). Forty-one sera negative for these specificities were tested by IP analysis of proteins ((35)S-methionine labeled K562 cell extract) and RNA (silver staining). Among 216 SSc patients analyzed, anti-topo I, ACA, and anti-RNAPIII were detected in 38% (81/216), 31% (67/216) and 7% (15/216), respectively. Among 41 sera negative for ACA, anti-topo I, and anti-RNAPIII and which were tested by IP, 14 were nucleolar stain-positive. Eight out of 14 (57%) showed anti-Th/To reactivity, but no anti-U3RNP was found. In comparison with ACA-positive patients, anti-Th/To-positive patients were younger (p = 0.0046) and more commonly were male (p = 0.0006). All 8 anti-Th/To-positive and all but one ACA-positive patients had lcSSc. Interstitial lung disease (ILD) and pericarditis were more frequent in anti-Th/To-positive patients. Anti-Th/To are common in antinucleolar antibody-positive Italian patients with SSc. Anti-Th/To and ACA patients had lcSSc, with excellent prognosis. The anti-Th/To group had frequent pericarditis and ILD, although impairment of pulmonary function appeared mild.
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To evaluate the frequency of malignancies in Italian patients with systemic sclerosis (SSc) and anti-RNA polymerase III (RNAP III), antitopoisomerase I (topo I), or anticentromere antibodies (ACA); and to characterize the temporal... more
To evaluate the frequency of malignancies in Italian patients with systemic sclerosis (SSc) and anti-RNA polymerase III (RNAP III), antitopoisomerase I (topo I), or anticentromere antibodies (ACA); and to characterize the temporal relationship between the 2 diseases, in order to confirm data suggesting a close temporal relationship between the onset of SSc and malignancy in American patients with anti-RNAP III antibodies. From a cohort of 466 consecutive SSc patients, 360 Italians with isolated positivity for anti-RNAP III (n = 16), anti-topo I (n = 101), or ACA (n = 243) were identified. Malignancy cases were divided according to their relationship with SSc onset into 3 categories: preceding, synchronous with, or metachronous to the onset of SSc (diagnosed more than 6 months before; 6 months before to 12 months after; and more than 12 months after onset of SSc, respectively). Malignancies were more frequent in the anti-RNAP III group (7/16 patients), than in the anti-topo I (11/101) and ACA groups (21/243) (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.001). This difference was accounted for by the number of patients with cancer synchronous to the onset of SSc (3/16 in the anti-RNAP III group vs 0/101 in the anti-topo I and 1/243 in the ACA group; p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.001), whereas neither the number of malignancies preceding nor those metachronous to the onset of SSc was significantly different between the groups. In a cohort of Italian patients with SSc we observed a significant association between malignancies synchronous to SSc onset and positivity for anti-RNAP III antibodies, similar to that described in American patients with SSc.
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... ANGELA CERIBELLI, MD,; ILARIA CAVAZZANA, MD,; PAOLO AIRÒ, MD and; FRANCO FRANCESCHINI, MD. Rheumatology Unit and Chair, Spedali Civili, Università degli Studi, Brescia, Italy. REFERENCES. ... Print ISSN: 0315-162X; Online ISSN:... more
... ANGELA CERIBELLI, MD,; ILARIA CAVAZZANA, MD,; PAOLO AIRÒ, MD and; FRANCO FRANCESCHINI, MD. Rheumatology Unit and Chair, Spedali Civili, Università degli Studi, Brescia, Italy. REFERENCES. ... Print ISSN: 0315-162X; Online ISSN: 1499-2752.
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Chilblain lupus erythematosus (CL) of Hutchinson is a subtype of lupus erythematosus (LE) characterized by erythematous lesions induced by cold, damp climates. A number of patients affected by CL eventually develop features of systemic... more
Chilblain lupus erythematosus (CL) of Hutchinson is a subtype of lupus erythematosus (LE) characterized by erythematous lesions induced by cold, damp climates. A number of patients affected by CL eventually develop features of systemic lupus erythematosus (SLE). We report here 9 patients with chilblain cutaneous lesions, 6 of them were affected by SLE and 2 by SCLE. The onset of CL preceded the diagnosis of LE, from 1 to 10 years in 3 cases, it was concurrent in one case and was subsequent in the remaining 4 cases. Raynaud&amp;amp;#39;s phenomenon and photosensitivity were other prominent clinical features in patients with CL. Nailfold capillaroscopy revealed pathological changes in every patient examined. ANA and anti-SSA/Ro antibodies were detected in all nine patients. Anti-SSB/La were detected in 2 cases, anti-Sm in one case, and anti-Sm and anti-RNP in a one case. Antibodies to dsDNA and complement consumption were found in the six patients with SLE. The fine specificity of anti-SSA/Ro was determined by immunoblotting: anti-60kD and anti-52 kD were detected in three sera, anti-60kD alone in 5 sera, while one serum did not blot. In conclusion, the present study suggests that chilblain LE is associated with SSA/Ro autoantibodies, as is SCLE, hypergammaglobulinemic purpura and neonatal lupus erythematosus.
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Research Interests: Echocardiography, Elasticity, Humans, Female, Male, and 4 moreHeart, Middle Aged, Aorta, and Case Control Studies
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Research Interests: Mental Health, Depression, Quality of life, Rheumatoid Arthritis, Translations, and 19 moreHumans, Health related Quality of Life, Systemic sclerosis, Female, Clinical, Male, Disease Severity, STEM (short form), Beck Depression Inventory, Clinical Sciences, General Health, Aged, Middle Aged, Questionnaires, Prognosis, Reproducibility of Results, Depressive Symptoms, Indexation, and Disease Activity
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To describe the clinical and immunologic features of 6 patients with rheumatic disease and Hepatitis C Virus (HCV) chronic infection, treated with anti-TNF alpha drugs. Six patients, with repeated positive serology for HCV infection, were... more
To describe the clinical and immunologic features of 6 patients with rheumatic disease and Hepatitis C Virus (HCV) chronic infection, treated with anti-TNF alpha drugs. Six patients, with repeated positive serology for HCV infection, were affected by Rheumatoid arthritis (RA) (4 cases), Psoriatic Arthritis (PsA) and Polymyositis in one case each. They started anti-TNFalpha treatment (Etanercept), due to a previous failure of combination of different immunosuppressants (Methotrexate, Sulfasalazine, Cyclosporine, Hydroxychloroquine). Patients (3 female and 3 males) showed a mean age at disease onset of 50.6 years (SD 14.5) and a mean disease duration of 12.5 years (SD: 8.8). Etanercept (dosage of 50 mg weekly) was continued for a median period of 14 months. Patients affected by RA and PsA achieved a good clinical response, with a significant reduction of DAS28 during treatment (p: 0.0001). No patient received any specific therapy for HCV infection. Elevated HCV-RNA titres were recorded in 5 cases at start of Etanercept. No significant increase was observed during anti-TNF alpha treatment. No cases of hepatic failure were recorded. Anti-TNF alpha therapy showed to be effective, safe and well tolerated in the setting of HCV infection.
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To define the clinical and immunologic profile of 9 patients with Sjögren&#39;s Syndrome (SS) and Hepatitis C virus (HCV) infection. 9 out of 305 patients with SS, diagnosed according to the criteria proposed in 2002, had repeated... more
To define the clinical and immunologic profile of 9 patients with Sjögren&#39;s Syndrome (SS) and Hepatitis C virus (HCV) infection. 9 out of 305 patients with SS, diagnosed according to the criteria proposed in 2002, had repeated positive serology for HCV. 9 female patients were studied. The mean age at onset of SS was 59 years, with a mean period of follow-up of 7.1 years. All the patients had glandular manifestations and they were all positive for dacryologic tests. Salivary gland biopsy was performed in 4 patients, all showing characteristic lymphocytic infiltrate. The main extraglandular features were arthralgias, photosensitivity, purpura, thyroiditis. All the patients were positive for anti-nuclear antibodies (ANA): 6 anti-Ro/SSA, 3 anti-Ro/SSA and anti-La/SSB positive. HCV-positive SS were compared with 296 patients with primary SS. They showed higher mean age (p=0.01), higher prevalence of photosensitivity (p=0.0266) and circulating cryoglobulins (p=0.0372). In primary SS, most patients had anti-Ro/SSA antibodies alone (49.8%) or associated to anti-La/SSB (46.5%). Five patients (1.8%) had other ANA specificities. A chronic HCV infection is concomitant in about 3% of patients with pSS. They differ from patients without HCV infection for the higher prevalence of photosensitivity and cryoglobulins, without clinical manifestations of cryoglobulinemia.
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The Antiphospholipid Syndrome (APS) is characterized by thrombosis and pregnancy loss, clinical events mediated by pathogenic anti-phospholipid autoantibodies (aPL). β2-glycoprotein I (β2GPI) is the major autoantigens recognized by aPL.... more
The Antiphospholipid Syndrome (APS) is characterized by thrombosis and pregnancy loss, clinical events mediated by pathogenic anti-phospholipid autoantibodies (aPL). β2-glycoprotein I (β2GPI) is the major autoantigens recognized by aPL. β2GPI is a cationic protein that binds to negatively charged surfaces such as those of apoptotic cells. This feature may lead to two major events: i) immunization with β2GPI fosters the Fc-receptor-mediated uptake by antigen presenting cells of apoptotic material decorated with β2GPI and the activation of β2GPI-specific T cells which in turn provide help to β2GPI-specific B cells for the production of anti-β2GPI; ii) apoptotic bodies decorated with β2GPI can be opsonized by anti-β2GPI and shifted towards a pro-inflammatory clearance by macrophages; epitope spread can occur with the generation of autoimmunity against nuclear autoantigens. In the presence of a predisposing genetic background and of a particular cytokine environment (type I interferons), the sequential emergence of autoantibodies can evolve into overt clinical disease. The spectrum of clinical phenotypes of the patients can be modulated by several factors affecting the pathogenicity of anti-β2GPI (e.g. domain specificity). We conclude that dying cells may play a dual role in APS: (I) as immunogen for the induction of aPL (etiology) and (II) as targets of aPL for the chronification of inflammation and the development of autoimmune diseases (pathology).
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Research Interests: Immunology, Electrocardiography, Prospective studies, Humans, Systemic Lupus Erythematosus, and 14 moreFemale, Male, Arthritis, Risk factors, Clinical Sciences, Newborn Infant, Prevalence, Public health systems and services research, Biological markers, Risk Factors, Ribonucleoproteins, Prospective Study, Autoantigens, and Bradycardia
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Page 1. AIDS RESEARCH AND HUMAN RETROVIRUSES Volume 6, Number 2, 1990 Mary Ann Liebert, Inc., Publishers Prevalence, Clinical, and Laboratory Features of Thrombocytopenia Among HIV-infected Individuals GIUSEPPE ...