Poster session 5: Innate and adaptive immunity, 2018
Systemic Lupus Erythematosus (SLE) has immune dysregulation, with lymphopenia being one of the mo... more Systemic Lupus Erythematosus (SLE) has immune dysregulation, with lymphopenia being one of the most frequent clinical findings, namely the CD4 T cells. It has been associated not only to higher risk of infections, but also to disease activity and risk of flares. The authors pretended to describe the prevalence of lymphopenia in SLE in a major centre of Immunology in Portugal. A retrospective analysis on the SLE patients was performed, with a longitudinal description of the subpopulations of lymphocytes, relating it with disease activity, organ involved, therapeutics and major infections. The sample had 48 patients, mainly constituted by females (85,4%), median age of 43 y. 62.5% had lymphopenia in the diagnosis. At the time of the most recent peripheral blood flow citometry 66,7% of the sample had lymphopenia, with different values of cytopenia according to lymphocytes subpopulations -T CD4: 77,4%; T CD8: 75%; B: 83,3%; NK: 91,7%. Severe T CD4 lymphopenia (below 200 uL) was present in 8,3% of the sample. 18,8% had severe flares (SLE-DAI Index) and 90% of these had low T CD4 counts (below 700 uL). The majority of patients with T CD4 under 200 uL were on severe flare. Higher frequency of corticosteroids and immunosuppressors, namely cyclophosphamide (12,5%) was observed on the patients. 17 patients of the sample had sequential citometry analysis and a correlation between lymphopenia and activity has not observed. 17 cumulative infections were described, the majority (70,6%) with lymphopenia. Although opportunistic infections (pulmonary aspergilosis, PML due to JC virus) were mainly seen on patients with T CD4 under 200 uL it was not mandatory this condition on this samplecryptococcal meningitis was described on a patient with 300 uL T CD4. Lymphopenia was present in the majority of active lupus and T CD4 seems to correlate with severe flare. Lymphopenia seems to be a bystander on the evolution of the disease. Despite rare, unpredictable infections can appear on patients with T CD4 counts superior to 200 uL.
As infeções nosocomiais são uma realidade constante no ambiente hospitalar. São responsáveis por ... more As infeções nosocomiais são uma realidade constante no ambiente hospitalar. São responsáveis por um elevado número de casos de infeções e são notoriamente difíceis de erradicar. Um dos motivos pelos quais o tratamento das infeções nosocomiais é difícil deve-se ao facto de muitas destas infeções serem causadas por biofilmes microbianos. Os biofilmes podem ser definidos como comunidades de micro-organismos que vivem aderidos a uma superfície e envoltos numa complexa mistura de compostos tais como proteínas, polissacáridos e DNA extracelular. O crescimento de micro-organismos sob a forma de biofilmes dificulta a sua erradicação, pois estas estruturas podem ser consideradas adaptações dos micro-organismos, de forma a continuar no hospedeiro. É especialmente relevante ter em consideração as infeções causadas por biofilmes no contexto hospitalar, pois podem necessitar de abordagens diferentes para a sua erradicação. Este artigo de revisão tem como objetivo reunir o conhecimento existente do papel dos biofilmes microbianos nas infeções nosocomiais.
The major purpose of this study was to evaluate alternative automated methods of collecting data ... more The major purpose of this study was to evaluate alternative automated methods of collecting data on health related quality of life (HR-QoL). In order to achieve this, we developed a study with the following objectives: (1) to evaluated the feasibility of electronic version in patients with different chronic pathologies of the immune system using Short Form 36version2 (SF-36v2), (2) to evaluate the construct validity of SF-36v2 using the electronic data capture, and (3) to compare electronic version questionnaires with paper questionnaires in terms of patients' acceptance, data quality, and reliability. Out-patients with chronic immune diseases (HIV infection, lupus, scleroderma, rheumatoid arthritis, Behçet and Sjögren), were randomly selected to completed electronic and paper SF-36v2 (n=50) before consultation in Clinical Immunology Unit, in Hospital Santo António-Centro Hospitalar do Porto (CI-HGSA). There were very high correlations in SF-36v2 responses (p< .001) between t...
Eosinophilic Fasciitis is a rare rheumatologic disease usually characterized by erythema and limb... more Eosinophilic Fasciitis is a rare rheumatologic disease usually characterized by erythema and limbs' pain, cutaneous induration and peripheral eosinophilia. However, it is important to consider Eosinophilic Fasciitis in differential diagnosis of others sclerodermalike syndromes. The authors present a case of a 52-year-old woman who presented to our hospital with a four months evolution of limb and trunk diffuse symmetrical cutaneous induration, thickening of the skin without joint involvement and no history of Raynaud's phenomenon. On physical examination, she had a grade 3 skin thickening in the involved segments. Laboratory studies revealed no peripheral eosinophilia. The biopsy showed fibrotic involvement of muscle and fascia, with a few inflammatory infiltrations of lymphocytes. She started prednisolone 1 mg/kg/day, with partial regression of cutaneous injuries. As Eosinophilic Fasciitis is so rare, its recognition can be difficult, especially when the patient is observed at a late stage where the peripheral eosinophilia and inflammatory infiltration in the histology are usually no longer present. A clinical history and a detailed physical examination, especially with the presence of orange peel skin, are the fundamental clues for the diagnosis.
The principle of treating-to-target has been successfully applied to many diseases outside rheuma... more The principle of treating-to-target has been successfully applied to many diseases outside rheumatology and more recently to rheumatoid arthritis. Identifying appropriate therapeutic targets and pursuing these systematically has led to improved care for patients with these diseases and useful guidance for healthcare providers and administrators. Thus, an initiative to evaluate possible therapeutic targets and develop treat-to-target guidance was believed to be highly appropriate in the management of systemic lupus erythematosus (SLE) patients as well. Specialists in rheumatology, nephrology, dermatology, internal medicine and clinical immunology, and a patient representative, contributed to this initiative. The majority convened on three occasions in 2012-2013. Twelve topics of critical importance were identified and a systematic literature review was performed. The results were condensed and reformulated as recommendations, discussed, modified and voted upon. The finalised bullet p...
Clinical and experimental immunology, Jan 24, 2017
FOXP3(+) regulatory T-cells (Tregs) are functionally deficient in Systemic Lupus Erythematosus (S... more FOXP3(+) regulatory T-cells (Tregs) are functionally deficient in Systemic Lupus Erythematosus (SLE), characterized by reduced surface CD25 (the IL-2 receptor alpha chain). Low-dose IL-2 therapy is a promising current approach to correct this defect. To elucidate the origins of the SLE Treg phenotype, we studied its role through developmentally defined Treg subsets in 45 SLE patients, 103 SLE-unaffected first-degree relatives and 61 unrelated healthy control subjects, and genetic association with the CD25-encoding IL2RA locus. We identified two separate, uncorrelated effects contributing to Treg CD25. (1) SLE patients and unaffected relatives remarkably shared CD25 reduction versus controls particularly in the developmentally earliest CD4(+) FOXP3(+) CD45RO(-) CD31(+) recent thymic emigrant Tregs. This first component effect influenced the proportions of circulating CD4(+) FOXP3(high) CD45RO(+) activated Tregs. (2) In contrast, patients and unaffected relatives sharply differed in t...
There is currently no approved treatment for primary Sjögren’s syndrome, a disease that primarily... more There is currently no approved treatment for primary Sjögren’s syndrome, a disease that primarily affects adult women. The difficulty in developing effective therapies is -in part- because of the heterogeneity in the clinical manifestation and pathophysiology of the disease. Finding common molecular signatures among patient subgroups could improve our understanding of disease etiology, and facilitate the development of targeted therapeutics. Here, we report, in a cross-sectional cohort, a molecular classification scheme for Sjögren’s syndrome patients based on the multi-omic profiling of whole blood samples from a European cohort of over 300 patients, and a similar number of age and gender-matched healthy volunteers. Using transcriptomic, genomic, epigenetic, cytokine expression and flow cytometry data, combined with clinical parameters, we identify four groups of patients with distinct patterns of immune dysregulation. The biomarkers we identify can be used by machine learning clas...
Rheumatoid arthritis (RA) is a chronic, systemic, inflammatory disorder of unknown etiology. It i... more Rheumatoid arthritis (RA) is a chronic, systemic, inflammatory disorder of unknown etiology. It is characterized by symmetric, polyarticular pain, swelling, morning stiffness, and fatigue. RA has a variable course, often with periods of exacerbations and, less frequently, true remissions. Outcomes are also variable, ranging from the rarely seen remitting disease to severe disease that brings disability and in some patients premature death. Without treatment, the majority of patients will experience progressive joint damage and, in some patients, this results in significant disability within just a few years. Improvement in health related quality of life is one of the most important goals in the management of rheumatoid arthritis and therefore must be pursued as a crucial aim in clinical practice. This paper draws from 49 qualitative interviews conducted with RA patients, before starting a program of Spa Therapy and aims to get an overview of their main RA related difficulties, worri...
Gastroenterology and Hepatology From Bed to Bench, 2016
Aim: The aim of this study was to review our experience with gestations in autoimmune hepatitis p... more Aim: The aim of this study was to review our experience with gestations in autoimmune hepatitis patients. Background: There are only limited data describing pregnancy in patients with autoimmune hepatitis. Patients and methods: Retrospective analysis of pregnancies with autoimmune hepatitis followed in Centro Hospitalar do Porto, Portugal in the last ten years. Results: We reported nine pregnancies in seven patients with autoimmune hepatitis. Two patients had documented liver cirrhosis prior to the pregnancy. In this study, 66.7% of patients were treated with azathioprine and 88.9% with prednisolone. Clinical improvements were observed in 11.1% of pregnancies and 22.2% exacerbations were diagnosed. There were six live births and two preterm deliveries (preterm delivery rate of 33%). We also report three first trimester miscarriages (early gestation miscarriage rate of 33%). There were no neonatal or maternal deaths. Conclusion: The favorable obstetric outcome is a realistic expectat...
62) mm, and LV end-systolic dimensions (LVESD) of 46.1 (30-55) mm. Regarding management, all pati... more 62) mm, and LV end-systolic dimensions (LVESD) of 46.1 (30-55) mm. Regarding management, all patients received pulse steroids, 7 were treated with IV cyclophosphamide, 3 started mycophenolate mofetil, and 1 patient underwent 3 sessions of immunoadsorption. Follow-up echocardiography after 3 months showed a mean LVEF of 42.8 (15-66)%, LVEDD of 52 (42-62) mm, and LVESD of 38.9 (22-55) mm. Regarding outcomes, 4 patients had a complete recovery and normalization of LVEF, all of them were females, 3 presented with cardiogenic shock and 1 with acute heart failure, with prompt initiation of immunosuppressive therapy. 2 patients became asymptomatic but achieved partial LVEF improvement. Mean duration to LVEF improvement in all patients was 2.7 (1-4) weeks. 4 patients failed to respond to treatment, three of them died within 3-months of LM diagnosis, including the 2 males and those on MHD, all 3 had biopsy-proven class IV LN. Conclusion It is crucial to recognize LM in the setting of acute heart failure & cardiogenic shock in SLE patients, as early diagnosis and prompt treatment with pulse steroids and other immunosuppressive drugs may achieve complete remission of myocarditis. Male gender with class IV LN biopsy proven or on MHD may be useful as predictors of poor prognosis.
The Israel Medical Association journal : IMAJ, 2016
The effects of balneotherapy on rheumatoid arthritis (RA) are still controversial partly due to p... more The effects of balneotherapy on rheumatoid arthritis (RA) are still controversial partly due to poor methodology used in randomized controlled trials, as reported in the international medical literature. To determine whether spa therapy plus pharmacological treatment offers any benefit in the management of RA as compared to pharmacological treatment alone. We conducted a prospective, controlled, unblinded randomly assigned study of patients with RA according to American College of Rheumatology criteria. Following the 2007 recommendations of AFRETH, the method designed for this study was "immediate treatment versus delayed treatment." All patients were followed at the Centro Hospitalar do Porto and each physician observed the same patients throughout the study. Patients continued with their usual medications and maintained their daily life activities at home, at leisure and/or in the workplace. The spa therapy group received spa treatments for 21 days at S. Jorge Spa-Santa ...
Clinical and experimental rheumatology, Jan 5, 2015
To investigate the role of endothelial dysfunction and angiogenesis vascular biomarkers as risk f... more To investigate the role of endothelial dysfunction and angiogenesis vascular biomarkers as risk factors and their predictive value for digital ulcers in systemic sclerosis patients. Endothelin-1 (ET-1), asymmetric dimethylarginine (ADMA), vascular endothelial growth factor (VEGF), endostatin and endoglin were measured in an observational prospective cohort of 77 SSc patients. The primary outcome was the occurrence of one or more new ischaemic digital ulcers during a planned 3-year follow-up. After the 3-year follow-up, 40 patients developed new digital ulcers. Logistic regression confirmed VEGF (HR 1.128, 95% CI 1.010-1.260, p=0.033) and ADMA (HR 0.995, 95% CI 0.991-0.998, p=0.006) as independent predictors of new digital ulcers. Patients with serum levels of ET-1>11.9pmol/ml (p<0.001) and VEGF<422.47 pg/ml (p=0.028) had significantly more DU in the 3-year follow-up. Although not significant, a trend towards increased serum levels of…
observed an incidence of ABH of 0.53%. 3 Several pathogenic factors are proposed, including repea... more observed an incidence of ABH of 0.53%. 3 Several pathogenic factors are proposed, including repeated minor trauma of oral mucosa (from hard or crispy food), the use of steroid-based inhalers for the treatment of asthma, dental procedure, endoscopy or local anaesthesia. 4,6 The association of ABH with systemic diseases such as hypertension, diabetes, rheumatoid arthritis, gastrointestinal disturbances, hyperuricemia and renal failure has been proposed but never confirmed. 3,4,6 However, as lesions of ABH can be easily confused with those occurring in many dermatological and systemic disorders, it is crucial to differentiate ABH from other more serious diseases which are the differential diagnosis, such as lichen planus pemphigoides, linear IgA bullous dermatosis, pemphigus, bullous pemphigoid, epidermolysis bullosa and haemostasis disorder. 2 ABH has not received much attention in the literature, and is not well known to many dermatologists and oral physicians. Thus, it is necessary to improve the knowledge of this disease (triggers, associated pathology, etc.) to shorten the diagnosis delay and reduce the risk of misdiagnosis.
To investigate whether CCR5 deletion is associated with susceptibility to Behçet's disease (B... more To investigate whether CCR5 deletion is associated with susceptibility to Behçet's disease (BD) in a Portuguese population. A total of 122 BD patients and 227 ethnically-matched controls were studied. Genotyping of the CCR5Δ32 polymorphisms was performed using polymerase chain reaction product sizing. No significant differences were observed in the allelic frequencies of CCR532 between patients and controls (OR=0.820; p=0.512). Stratification for gender and for the presence of HLA-B*51 did not reveal any significant differences. These results indicate that CCR5Δ32 is unlikely to contribute to susceptibility to BD in Portuguese patients. This may be explained by the known functional redundancy of this signalling system.
Poster session 5: Innate and adaptive immunity, 2018
Systemic Lupus Erythematosus (SLE) has immune dysregulation, with lymphopenia being one of the mo... more Systemic Lupus Erythematosus (SLE) has immune dysregulation, with lymphopenia being one of the most frequent clinical findings, namely the CD4 T cells. It has been associated not only to higher risk of infections, but also to disease activity and risk of flares. The authors pretended to describe the prevalence of lymphopenia in SLE in a major centre of Immunology in Portugal. A retrospective analysis on the SLE patients was performed, with a longitudinal description of the subpopulations of lymphocytes, relating it with disease activity, organ involved, therapeutics and major infections. The sample had 48 patients, mainly constituted by females (85,4%), median age of 43 y. 62.5% had lymphopenia in the diagnosis. At the time of the most recent peripheral blood flow citometry 66,7% of the sample had lymphopenia, with different values of cytopenia according to lymphocytes subpopulations -T CD4: 77,4%; T CD8: 75%; B: 83,3%; NK: 91,7%. Severe T CD4 lymphopenia (below 200 uL) was present in 8,3% of the sample. 18,8% had severe flares (SLE-DAI Index) and 90% of these had low T CD4 counts (below 700 uL). The majority of patients with T CD4 under 200 uL were on severe flare. Higher frequency of corticosteroids and immunosuppressors, namely cyclophosphamide (12,5%) was observed on the patients. 17 patients of the sample had sequential citometry analysis and a correlation between lymphopenia and activity has not observed. 17 cumulative infections were described, the majority (70,6%) with lymphopenia. Although opportunistic infections (pulmonary aspergilosis, PML due to JC virus) were mainly seen on patients with T CD4 under 200 uL it was not mandatory this condition on this samplecryptococcal meningitis was described on a patient with 300 uL T CD4. Lymphopenia was present in the majority of active lupus and T CD4 seems to correlate with severe flare. Lymphopenia seems to be a bystander on the evolution of the disease. Despite rare, unpredictable infections can appear on patients with T CD4 counts superior to 200 uL.
As infeções nosocomiais são uma realidade constante no ambiente hospitalar. São responsáveis por ... more As infeções nosocomiais são uma realidade constante no ambiente hospitalar. São responsáveis por um elevado número de casos de infeções e são notoriamente difíceis de erradicar. Um dos motivos pelos quais o tratamento das infeções nosocomiais é difícil deve-se ao facto de muitas destas infeções serem causadas por biofilmes microbianos. Os biofilmes podem ser definidos como comunidades de micro-organismos que vivem aderidos a uma superfície e envoltos numa complexa mistura de compostos tais como proteínas, polissacáridos e DNA extracelular. O crescimento de micro-organismos sob a forma de biofilmes dificulta a sua erradicação, pois estas estruturas podem ser consideradas adaptações dos micro-organismos, de forma a continuar no hospedeiro. É especialmente relevante ter em consideração as infeções causadas por biofilmes no contexto hospitalar, pois podem necessitar de abordagens diferentes para a sua erradicação. Este artigo de revisão tem como objetivo reunir o conhecimento existente do papel dos biofilmes microbianos nas infeções nosocomiais.
The major purpose of this study was to evaluate alternative automated methods of collecting data ... more The major purpose of this study was to evaluate alternative automated methods of collecting data on health related quality of life (HR-QoL). In order to achieve this, we developed a study with the following objectives: (1) to evaluated the feasibility of electronic version in patients with different chronic pathologies of the immune system using Short Form 36version2 (SF-36v2), (2) to evaluate the construct validity of SF-36v2 using the electronic data capture, and (3) to compare electronic version questionnaires with paper questionnaires in terms of patients' acceptance, data quality, and reliability. Out-patients with chronic immune diseases (HIV infection, lupus, scleroderma, rheumatoid arthritis, Behçet and Sjögren), were randomly selected to completed electronic and paper SF-36v2 (n=50) before consultation in Clinical Immunology Unit, in Hospital Santo António-Centro Hospitalar do Porto (CI-HGSA). There were very high correlations in SF-36v2 responses (p< .001) between t...
Eosinophilic Fasciitis is a rare rheumatologic disease usually characterized by erythema and limb... more Eosinophilic Fasciitis is a rare rheumatologic disease usually characterized by erythema and limbs' pain, cutaneous induration and peripheral eosinophilia. However, it is important to consider Eosinophilic Fasciitis in differential diagnosis of others sclerodermalike syndromes. The authors present a case of a 52-year-old woman who presented to our hospital with a four months evolution of limb and trunk diffuse symmetrical cutaneous induration, thickening of the skin without joint involvement and no history of Raynaud's phenomenon. On physical examination, she had a grade 3 skin thickening in the involved segments. Laboratory studies revealed no peripheral eosinophilia. The biopsy showed fibrotic involvement of muscle and fascia, with a few inflammatory infiltrations of lymphocytes. She started prednisolone 1 mg/kg/day, with partial regression of cutaneous injuries. As Eosinophilic Fasciitis is so rare, its recognition can be difficult, especially when the patient is observed at a late stage where the peripheral eosinophilia and inflammatory infiltration in the histology are usually no longer present. A clinical history and a detailed physical examination, especially with the presence of orange peel skin, are the fundamental clues for the diagnosis.
The principle of treating-to-target has been successfully applied to many diseases outside rheuma... more The principle of treating-to-target has been successfully applied to many diseases outside rheumatology and more recently to rheumatoid arthritis. Identifying appropriate therapeutic targets and pursuing these systematically has led to improved care for patients with these diseases and useful guidance for healthcare providers and administrators. Thus, an initiative to evaluate possible therapeutic targets and develop treat-to-target guidance was believed to be highly appropriate in the management of systemic lupus erythematosus (SLE) patients as well. Specialists in rheumatology, nephrology, dermatology, internal medicine and clinical immunology, and a patient representative, contributed to this initiative. The majority convened on three occasions in 2012-2013. Twelve topics of critical importance were identified and a systematic literature review was performed. The results were condensed and reformulated as recommendations, discussed, modified and voted upon. The finalised bullet p...
Clinical and experimental immunology, Jan 24, 2017
FOXP3(+) regulatory T-cells (Tregs) are functionally deficient in Systemic Lupus Erythematosus (S... more FOXP3(+) regulatory T-cells (Tregs) are functionally deficient in Systemic Lupus Erythematosus (SLE), characterized by reduced surface CD25 (the IL-2 receptor alpha chain). Low-dose IL-2 therapy is a promising current approach to correct this defect. To elucidate the origins of the SLE Treg phenotype, we studied its role through developmentally defined Treg subsets in 45 SLE patients, 103 SLE-unaffected first-degree relatives and 61 unrelated healthy control subjects, and genetic association with the CD25-encoding IL2RA locus. We identified two separate, uncorrelated effects contributing to Treg CD25. (1) SLE patients and unaffected relatives remarkably shared CD25 reduction versus controls particularly in the developmentally earliest CD4(+) FOXP3(+) CD45RO(-) CD31(+) recent thymic emigrant Tregs. This first component effect influenced the proportions of circulating CD4(+) FOXP3(high) CD45RO(+) activated Tregs. (2) In contrast, patients and unaffected relatives sharply differed in t...
There is currently no approved treatment for primary Sjögren’s syndrome, a disease that primarily... more There is currently no approved treatment for primary Sjögren’s syndrome, a disease that primarily affects adult women. The difficulty in developing effective therapies is -in part- because of the heterogeneity in the clinical manifestation and pathophysiology of the disease. Finding common molecular signatures among patient subgroups could improve our understanding of disease etiology, and facilitate the development of targeted therapeutics. Here, we report, in a cross-sectional cohort, a molecular classification scheme for Sjögren’s syndrome patients based on the multi-omic profiling of whole blood samples from a European cohort of over 300 patients, and a similar number of age and gender-matched healthy volunteers. Using transcriptomic, genomic, epigenetic, cytokine expression and flow cytometry data, combined with clinical parameters, we identify four groups of patients with distinct patterns of immune dysregulation. The biomarkers we identify can be used by machine learning clas...
Rheumatoid arthritis (RA) is a chronic, systemic, inflammatory disorder of unknown etiology. It i... more Rheumatoid arthritis (RA) is a chronic, systemic, inflammatory disorder of unknown etiology. It is characterized by symmetric, polyarticular pain, swelling, morning stiffness, and fatigue. RA has a variable course, often with periods of exacerbations and, less frequently, true remissions. Outcomes are also variable, ranging from the rarely seen remitting disease to severe disease that brings disability and in some patients premature death. Without treatment, the majority of patients will experience progressive joint damage and, in some patients, this results in significant disability within just a few years. Improvement in health related quality of life is one of the most important goals in the management of rheumatoid arthritis and therefore must be pursued as a crucial aim in clinical practice. This paper draws from 49 qualitative interviews conducted with RA patients, before starting a program of Spa Therapy and aims to get an overview of their main RA related difficulties, worri...
Gastroenterology and Hepatology From Bed to Bench, 2016
Aim: The aim of this study was to review our experience with gestations in autoimmune hepatitis p... more Aim: The aim of this study was to review our experience with gestations in autoimmune hepatitis patients. Background: There are only limited data describing pregnancy in patients with autoimmune hepatitis. Patients and methods: Retrospective analysis of pregnancies with autoimmune hepatitis followed in Centro Hospitalar do Porto, Portugal in the last ten years. Results: We reported nine pregnancies in seven patients with autoimmune hepatitis. Two patients had documented liver cirrhosis prior to the pregnancy. In this study, 66.7% of patients were treated with azathioprine and 88.9% with prednisolone. Clinical improvements were observed in 11.1% of pregnancies and 22.2% exacerbations were diagnosed. There were six live births and two preterm deliveries (preterm delivery rate of 33%). We also report three first trimester miscarriages (early gestation miscarriage rate of 33%). There were no neonatal or maternal deaths. Conclusion: The favorable obstetric outcome is a realistic expectat...
62) mm, and LV end-systolic dimensions (LVESD) of 46.1 (30-55) mm. Regarding management, all pati... more 62) mm, and LV end-systolic dimensions (LVESD) of 46.1 (30-55) mm. Regarding management, all patients received pulse steroids, 7 were treated with IV cyclophosphamide, 3 started mycophenolate mofetil, and 1 patient underwent 3 sessions of immunoadsorption. Follow-up echocardiography after 3 months showed a mean LVEF of 42.8 (15-66)%, LVEDD of 52 (42-62) mm, and LVESD of 38.9 (22-55) mm. Regarding outcomes, 4 patients had a complete recovery and normalization of LVEF, all of them were females, 3 presented with cardiogenic shock and 1 with acute heart failure, with prompt initiation of immunosuppressive therapy. 2 patients became asymptomatic but achieved partial LVEF improvement. Mean duration to LVEF improvement in all patients was 2.7 (1-4) weeks. 4 patients failed to respond to treatment, three of them died within 3-months of LM diagnosis, including the 2 males and those on MHD, all 3 had biopsy-proven class IV LN. Conclusion It is crucial to recognize LM in the setting of acute heart failure & cardiogenic shock in SLE patients, as early diagnosis and prompt treatment with pulse steroids and other immunosuppressive drugs may achieve complete remission of myocarditis. Male gender with class IV LN biopsy proven or on MHD may be useful as predictors of poor prognosis.
The Israel Medical Association journal : IMAJ, 2016
The effects of balneotherapy on rheumatoid arthritis (RA) are still controversial partly due to p... more The effects of balneotherapy on rheumatoid arthritis (RA) are still controversial partly due to poor methodology used in randomized controlled trials, as reported in the international medical literature. To determine whether spa therapy plus pharmacological treatment offers any benefit in the management of RA as compared to pharmacological treatment alone. We conducted a prospective, controlled, unblinded randomly assigned study of patients with RA according to American College of Rheumatology criteria. Following the 2007 recommendations of AFRETH, the method designed for this study was "immediate treatment versus delayed treatment." All patients were followed at the Centro Hospitalar do Porto and each physician observed the same patients throughout the study. Patients continued with their usual medications and maintained their daily life activities at home, at leisure and/or in the workplace. The spa therapy group received spa treatments for 21 days at S. Jorge Spa-Santa ...
Clinical and experimental rheumatology, Jan 5, 2015
To investigate the role of endothelial dysfunction and angiogenesis vascular biomarkers as risk f... more To investigate the role of endothelial dysfunction and angiogenesis vascular biomarkers as risk factors and their predictive value for digital ulcers in systemic sclerosis patients. Endothelin-1 (ET-1), asymmetric dimethylarginine (ADMA), vascular endothelial growth factor (VEGF), endostatin and endoglin were measured in an observational prospective cohort of 77 SSc patients. The primary outcome was the occurrence of one or more new ischaemic digital ulcers during a planned 3-year follow-up. After the 3-year follow-up, 40 patients developed new digital ulcers. Logistic regression confirmed VEGF (HR 1.128, 95% CI 1.010-1.260, p=0.033) and ADMA (HR 0.995, 95% CI 0.991-0.998, p=0.006) as independent predictors of new digital ulcers. Patients with serum levels of ET-1>11.9pmol/ml (p<0.001) and VEGF<422.47 pg/ml (p=0.028) had significantly more DU in the 3-year follow-up. Although not significant, a trend towards increased serum levels of…
observed an incidence of ABH of 0.53%. 3 Several pathogenic factors are proposed, including repea... more observed an incidence of ABH of 0.53%. 3 Several pathogenic factors are proposed, including repeated minor trauma of oral mucosa (from hard or crispy food), the use of steroid-based inhalers for the treatment of asthma, dental procedure, endoscopy or local anaesthesia. 4,6 The association of ABH with systemic diseases such as hypertension, diabetes, rheumatoid arthritis, gastrointestinal disturbances, hyperuricemia and renal failure has been proposed but never confirmed. 3,4,6 However, as lesions of ABH can be easily confused with those occurring in many dermatological and systemic disorders, it is crucial to differentiate ABH from other more serious diseases which are the differential diagnosis, such as lichen planus pemphigoides, linear IgA bullous dermatosis, pemphigus, bullous pemphigoid, epidermolysis bullosa and haemostasis disorder. 2 ABH has not received much attention in the literature, and is not well known to many dermatologists and oral physicians. Thus, it is necessary to improve the knowledge of this disease (triggers, associated pathology, etc.) to shorten the diagnosis delay and reduce the risk of misdiagnosis.
To investigate whether CCR5 deletion is associated with susceptibility to Behçet's disease (B... more To investigate whether CCR5 deletion is associated with susceptibility to Behçet's disease (BD) in a Portuguese population. A total of 122 BD patients and 227 ethnically-matched controls were studied. Genotyping of the CCR5Δ32 polymorphisms was performed using polymerase chain reaction product sizing. No significant differences were observed in the allelic frequencies of CCR532 between patients and controls (OR=0.820; p=0.512). Stratification for gender and for the presence of HLA-B*51 did not reveal any significant differences. These results indicate that CCR5Δ32 is unlikely to contribute to susceptibility to BD in Portuguese patients. This may be explained by the known functional redundancy of this signalling system.
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Papers by Carlos Vasconcelos