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...