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OBJECTIVES: To assess agreement between the 2021 Definition Of Remission In SLE (DORIS) and physician-judged lupus activity. METHODS: A cross-sectional analysis was conducted of data from a Spanish prospective multicentre study of SLE patients. We applied the 2021 DORIS criteria and assessed whether remission status based on this definition agreed with remission as per physician clinical judgement and reasons for disagreement between them. RESULTS: Out of 508 patients [92% women; mean age (s.d.): 50.4 years (13.7)] studied, 267 (54.4%) met the criteria for 2021 DORIS remission. Based on physicians' judgement, 277 (55.9%) patients were classified as in remission or serologically active clinically quiescent (SACQ). The overall rate of agreement between these assessments was 81.2% (95% CI: 79.9, 82.9%) with a Cohen's kappa of 0.62 (0.55-0.69). Overall, 46 (9.1%) patients were classified as in remission/SACQ by rheumatologists but did not meet the 2021 DORIS criteria for remission. The main reasons for discrepancies were a clinical SLE Disease Activity Index (cSLEDAI) score >0 in 39 patients, a Physician Global Assessment score >0.5 in five patients, and prednisone >5 mg/day in another five patients. CONCLUSIONS: The 2021 DORIS remission is an achievable target in clinical practice. There is substantial agreement between the DORIS definition and physician-judged remission. The discordance was mainly due to physicians classifying some patients with ongoing mild disease activity as in remission. Thus, the standardized DORIS definition should be used to define the target in a treat-to-target strategy for the management of SLE.
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Julgamento , Lúpus Eritematoso Sistêmico , Humanos , Feminino , Masculino , Estudos Prospectivos , Estudos Transversais , Reumatologistas , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Índice de Gravidade de Doença , Indução de RemissãoRESUMO
Advanced glycation end-products (AGEs) may play a relevant role as inducers in the chronic inflammatory pathway present in immune-mediated diseases, such as systemic lupus erythematosus (SLE). AGEs concentrations have been associated, with discrepant results to date, with some parameters such as disease activity or accrual damage, suggesting their potential usefulness as biomarkers of the disease. Our objectives are to confirm differences in AGEs levels measured by cutaneous autofluorescence between SLE patients and healthy controls (HC) and to study their correlation with various disease parameters. Cross-sectional study, where AGEs levels were measured by skin autofluorescence, and SLE patients' data were compared with those of sex- and age-matched HC in a 1:3 proportion through a multiple linear regression model. Associations of AGEs levels with demographic and clinical data were analyzed through ANOVA tests. Both analyses were adjusted for confounders. AGEs levels in SLE patients were significantly higher than in HC (p < 0.001). We found statistically significant positive associations with SLE disease activity index (SLEDAI) and damage index (SDI), physician and patient global assessment, C-reactive protein, leukocyturia, complement C4, IL-6 and oral ulcers. We also found a negative statistically significant association with current positivity of anti-nuclear and anti-Ro60 antibodies. AGEs seem to have a contribution in LES pathophysiology, being associated with activity and damage and having a role as a new management and prognosis biomarker in this disease. The association with specific antibodies and disease manifestations may indicate a specific clinical phenotype related to higher or lower AGEs levels.
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Lúpus Eritematoso Sistêmico , Humanos , Estudos Transversais , Biomarcadores , Complemento C4 , Índice de Gravidade de Doença , Produtos Finais de Glicação AvançadaRESUMO
OBJECTIVES: To apply the lupus low disease activity state (LLDAS) definition within a large cohort of patients and to assess the agreement between the LLDAS and the physician's subjective evaluation of lupus activity. METHODS: We conducted a cross-sectional analysis of a prospective multicentre study of SLE patients. We applied the LLDAS and assessed whether there was agreement with the clinical status according to the physician's opinion. RESULTS: A total of 508 patients [92% women; mean age 50.4 years (s.d. 3.7)] were recruited and 304 (62.7%) patients were in the LLDAS. According to physician assessment, 430 (86.1%) patients were classified as remission or low activity. Overall agreement between both evaluations was 71.4% (95% CI: 70.1, 70.5) with a Cohen's κ of 0.3 [interquartile range (IQR) 0.22-0.37]. Most cases (96.1%) in the LLDAS were classified as remission or low activity by the expert. Of the patients who did not fulfil the LLDAS, 126 (70.4%) were classified as having remission/low disease activity. The main reasons for these discrepancies were the presence of new manifestations compared with the previous visit and a SLEDAI 2K score >4, mainly based on serological activity. CONCLUSIONS: Almost two-thirds of SLE patients were in the LLDAS. There was a fair correlation between the LLDAS and the physician's evaluation. This agreement improves for patients fulfilling the LLDAS criteria. The discordance between both at defining lupus low activity, the demonstrated association of the LLDAS with better outcomes and the fact that the LLDAS is more stringent than the physician's opinion imply that we should use the LLDAS as a treat-to-target goal.
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Prova Pericial , Lúpus Eritematoso Sistêmico , Humanos , Feminino , Pessoa de Meia-Idade , Masculino , Estudos Transversais , Índice de Gravidade de DoençaRESUMO
BACKGROUND: Systemic lupus erythematosus (SLE) is an autoimmune multisystemic disease with a wide variety of clinical manifestations. One of its symptoms, associated to high morbidity, is serositis. Its prevalence ranges between 11% and 54%, and little is known about factors associated to this manifestation. The aim of this study is to determine the prevalence of serositis in SLE patients visited at the outpatient Lupus Unit of the Hospital del Mar and identify risk factors that can be used as predictors of this manifestation. METHODS: A retrospective case-control study was performed based on the review of 297 medical records of SLE patients. Twenty-eight patients were identified to have suffered serositis (cases) and were age- and sex-matched with 2 controls with SLE without serositis. RESULTS: The overall prevalence of serositis in our cohort was 9.42%, being higher in men than in women, 30% versus 7.9% (p = 0.001, 95% CI: 1.7-42.4%). In 40.7%, it was the first manifestation of the disease. When looking for serositis-associated factors, an association was found with anti-dsDNA antibodies measured by the Crithidia method (p = 0.016), and different measures of corticosteroids, where cases had required higher maximum doses and more pulses than controls throughout the disease, although this last correlation was lost when adjusting for confounding variables as nephritis and arthritis. Cases also received more mycophenolic acid (p = 0.021) and, marginally, more belimumab (p = 0.056). CONCLUSION: The overall prevalence of serositis was 9.42%, being significantly higher in men (30%). Therefore, male gender constitutes a risk factor for serositis, and almost one third of men will develop this manifestation, so greater awareness is required in SLE men. CrithidiaDNA+ was also identified as a risk factor, and it should be determined in all SLE patients. Cases significantly received more corticosteroid pulses and higher maximum doses in relation to other SLE severe manifestations, which could imply a more aggressive form of SLE in patients with serositis.
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Lúpus Eritematoso Sistêmico , Serosite , Anticorpos Antinucleares , Estudos de Casos e Controles , Feminino , Humanos , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Lúpus Eritematoso Sistêmico/epidemiologia , Masculino , Prevalência , Estudos Retrospectivos , Fatores de Risco , Serosite/epidemiologiaRESUMO
INTRODUCTION: Immune thrombocytopenia (ITP) is a potentially severe manifestation of systemic lupus erythematosus (SLE) reported in 7-40% of SLE patients. ITP has been associated with a higher risk of organ damage and mortality. OBJECTIVES: To describe which factors are associated with the presence of ITP in SLE patients. METHODS: Retrospective case-control study. Cases were defined as SLE patients who had ever developed ITP and were sex- and age-matched with two controls. A predictive model was constructed to identify SLE patients who were at risk of developing ITP. RESULTS: ITP prevalence in our SLE cohort was 8.35%. Cases had a higher frequency of hemolytic anemia, while controls had a higher prevalence of arthritis at SLE diagnosis. During SLE progression, cases tested positive for anticardiolipin, anti-ß2-glycoprotein 1, and lupus anticoagulant antibodies more frequently. Cases received mycophenolic acid and azathioprine more often than controls and had a higher SLICC/ACR score. The model demonstrated a sensitivity of 87.53%, a positive predictive value of 81.92%, a specificity of 80.50%, area under the curve of 83.92%, a F1 of 83% and an overall accuracy of 83.68%. The variables that best explain the model were hemolytic anemia, arthritis, oral ulcers, Raynaud's phenomenon, low C4, low CH50, anticardiolipin and anti-ß2GP1 antibodies. CONCLUSION: SLE patients who develop ITP have a distinct phenotype characterized by more hemolytic anemia and less arthritis at SLE onset, and higher prevalence of antiphospholipid syndrome antibodies during SLE progression. This phenotype is associated with heightened organ damage and the need for more intensive therapies and stricter follow-up. Our predictive model has demonstrated an impressive ability to identify SLE patients at risk of developing ITP.
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Lúpus Eritematoso Sistêmico , Púrpura Trombocitopênica Idiopática , Humanos , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/epidemiologia , Feminino , Estudos Retrospectivos , Masculino , Adulto , Prevalência , Estudos de Casos e Controles , Medição de Risco , Fatores de Risco , Púrpura Trombocitopênica Idiopática/epidemiologia , Púrpura Trombocitopênica Idiopática/etiologia , Pessoa de Meia-Idade , Adulto JovemRESUMO
It has been postulated that advanced glycation end products (AGEs) and their soluble receptor (sRAGE) may play a relevant role as inducers in the chronic inflammatory pathway in various conditions, among them, in immune-mediated diseases such as systemic lupus erythematosus (SLE). However, previous studies show conflicting results about their association with SLE characteristics and their usefulness as disease biomarkers. We aimed to study the association of specific serum AGEs (pentosidine, Nξ-(carboxymethyl)lysine (CML), Nξ-(carboxyethyl)lysine (CEL)), sRAGE levels and AGEs (specific serum AGEs and skin AGEs) to sRAGE ratios with various disease parameters, in order to clarify their potential as new biomarkers in SLE and to study their relationship with cardiovascular disease (CVD). To this aim, serum pentosidine, CML, CEL and sRAGE were measured via ELISA, and skin AGEs levels were measured by skin autofluorescence. Correlations of pentosidine levels with demographic and clinical data, indexes of activity, accrual damage and patient-reported outcomes were analyzed through multiple linear regression models, while correlations of the rest of the AGEs, sRAGE and AGE to sRAGE ratios (non-normal) were analyzed using both an OLS regression model and a GML. All of the analyses were adjusted for confounders. A total of 119 SLE patients were recruited. Serum AGEs and sRAGEs were significantly associated with SLE activity indexes and/or demographic or disease characteristics: pentosidine with pulmonary manifestations; CML with anti-dsDNA antibodies, IL-6, disease duration and non-Caucasian ethnicities; CEL with anti-dsDNA antibodies, IL-6 and accumulated number of manifestations; and sRAGE with male gender, photosensitivity and being on specific immunosuppressants. These results suggest that the AGE-sRAGE axis may serve as a novel biomarker for managing and prognosticating this disease. Its correlation with certain antibodies, demographics and disease presentations may indicate a distinct clinical phenotype associated with varying levels of AGEs and/or sRAGE. The significance of specific AGE/sRAGE ratios, introduced in this study for the first time, warrants additional investigation in forthcoming research. Our study did not confirm the link between serum AGEs and CVD, which merits further exploration through studies designed for this specific purpose.
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OBJECTIVE: To develop an improved score for prediction of severe infection in patients with systemic lupus erythematosus (SLE), namely, the SLE Severe Infection Score-Revised (SLESIS-R) and to validate it in a large multicentre lupus cohort. METHODS: We used data from the prospective phase of RELESSER (RELESSER-PROS), the SLE register of the Spanish Society of Rheumatology. A multivariable logistic model was constructed taking into account the variables already forming the SLESIS score, plus all other potential predictors identified in a literature review. Performance was analysed using the C-statistic and the area under the receiver operating characteristic curve (AUROC). Internal validation was carried out using a 100-sample bootstrapping procedure. ORs were transformed into score items, and the AUROC was used to determine performance. RESULTS: A total of 1459 patients who had completed 1 year of follow-up were included in the development cohort (mean age, 49±13 years; 90% women). Twenty-five (1.7%) had experienced ≥1 severe infection. According to the adjusted multivariate model, severe infection could be predicted from four variables: age (years) ≥60, previous SLE-related hospitalisation, previous serious infection and glucocorticoid dose. A score was built from the best model, taking values from 0 to 17. The AUROC was 0.861 (0.777-0.946). The cut-off chosen was ≥6, which exhibited an accuracy of 85.9% and a positive likelihood ratio of 5.48. CONCLUSIONS: SLESIS-R is an accurate and feasible instrument for predicting infections in patients with SLE. SLESIS-R could help to make informed decisions on the use of immunosuppressants and the implementation of preventive measures.
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Lúpus Eritematoso Sistêmico , Humanos , Feminino , Adulto , Pessoa de Meia-Idade , Masculino , Lúpus Eritematoso Sistêmico/complicações , Estudos Prospectivos , Imunossupressores , Modelos LogísticosRESUMO
INTRODUCTION AND AIMS: Cardiac involvement in systemic sclerosis (SS) is frequently silent and a major cause of mortality in these patients. This work aims to study the prevalence and associations of left ventricular dysfunction (LVD) and arrhythmias in SS. METHODS AND RESULTS: Prospective study of SS patients (n=36), excluding those with symptoms of (or) cardiac disease, pulmonary arterial hypertension or cardiovascular risk factors (CVRF). A clinical, analytical, electrocardiogram (EKG), Holter, and echocardiogram with global longitudinal strain (GLS) assessment were performed. Arrhythmias were classified into clinically significant arrhythmias (CSA) and non-significant. Twenty-eight percent had left ventricular diastolic dysfunction (LVDD), 22% LV systolic dysfunction (LVSD) according to the GLS, 11.1% both, and 16.7% cardiac dysautonomia. Fifty percent presented alterations by EKG (44% CSA), 55.6% by Holter (75% CSA) and 8.3% CSA by both. An association was found between the elevation of troponin T (TnTc) and CSA and between the elevation of both NT-proBNP and TnTc with LVDD. CONCLUSIONS: We found a higher prevalence of LVSD than in the literature, detected by GLS and being 10 times higher than that detected by LVEF, which justifies the need to incorporate this technique in the routine evaluation of these patients. The association of TnTc and NT-proBNP with LVDD suggests that they can be used as minimally invasive biomarkers of this affectation. The absence of correlation between LVD and CSA indicates that the arrhythmias could be due, not only to a supposed structural alteration of the myocardium, but to an independent and early cardiac involvement, which should be actively investigated even in asymptomatic patients without CVRF.
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Escleroderma Sistêmico , Disfunção Ventricular Esquerda , Humanos , Estudos Prospectivos , Disfunção Ventricular Esquerda/etiologia , Disfunção Ventricular Esquerda/epidemiologia , Coração , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/epidemiologia , Arritmias Cardíacas/etiologia , Escleroderma Sistêmico/complicações , Função Ventricular Esquerda , Volume SistólicoRESUMO
Isolated extrapulmonary involvement in sarcoidosis is uncommon and reported in 5-9% of systemic sarcoidosis, this constitutes a clinical challenge due to its extensive differential diagnosis. Extrapulmonary sarcoidosis affecting more than three organs is rarely reported and there are scarce literature data published on diagnosis, clinical course and management in those cases. We hereby discuss a case of a 41-year-old female with systemic non-pulmonary sarcoidosis affecting lacrimal gland, peripheral lymph nodes, parotid gland and the liver.
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Sarcoidose , Adulto , Diagnóstico Diferencial , Feminino , Humanos , Sarcoidose/complicações , Sarcoidose/diagnósticoRESUMO
OBJECTIVES: To describe the methods of the Spanish Registry of patients with idiopathic inflammatory myopathy (IIM) (Myo-Spain), as well as its strengths and limitations. The main objective of the project is to analyse the evolution and clinical management of a cohort of patients with IIM. METHODS: Observational, longitudinal, ambispective and multicentre study of a cohort of patients with IIM seen in rheumatology units in Spain. All patients with a diagnosis of IMM will be included in the regular follow-up of the participating centres, regardless of age on initiation of the process. Incident cases will be all patients who at the beginning of the study have been diagnosed for less than 12 months and prevalent cases for more than 12 months. The registry will include data from the visit at baseline, one year and two years. Socio-demographic, clinical, analytical variables, complications, comorbidities, association with other rheumatic diseases, hospital admissions, mortality and treatments will be collected. In addition, indices, scales and questionnaires of activity, muscle involvement, damage, disability, and quality of life will be determined. The recruitment period will be 23 months. The purpose is to obtain a cohort of 400 patients with IMM. CONCLUSIONS: Myo-Spain registry provides the opportunity to develop a cohort of incident and prevalent patients with IMM in Spain. Myo-Spain will be able to assess in detail the clinical characteristics of the disease at different times. The comprehensive information collected during the visits is expected to provide a broad source of data for future analysis.
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Miosite , Reumatologia , Humanos , Miosite/diagnóstico , Miosite/epidemiologia , Miosite/terapia , Qualidade de Vida , Sistema de Registros , Espanha/epidemiologiaRESUMO
The neuropsychiatric involvement in systemic lupus erythematosus (NPSLE) is a challenge for clinicians, both at a diagnostic and therapeutic level. Although in 1999 the American College of Rheumatology (ACR) proposed a set of definitions for 19 NPSLE syndromes, with the intention of homogenizing the terminology for research purposes and clinical practice, the prevalence of NPSLE varies widely according to different series and is estimated to be between 37 and 95%. This is due to multiple factors such as the unalike definitions used, the diverse design of the studies, type of population, race, type and severity of symptoms, and follow-up of the different cohorts of patients with SLE. In recent years, some authors have tried excluding minor neuropsychiatric manifestations in order to try to reduce this wide variation in the prevalence of NPSLE since they are very prevalent in the general population; others authors have developed various models for the attribution of neuropsychiatric events to SLE that can assist clinicians in this diagnostic process, and finally, some authors developed and validated in 2014 a new algorithm based on the definitions of the ACR that includes the evaluation of the patient's lupus activity together with imaging techniques and the analysis of cerebrospinal fluid (CSF), with the aim of trying to differentiate the true neuropsychiatric manifestations attributable to SLE. In 2010, the European League Against Rheumatism (EULAR) developed recommendations for the management of NPSLE. We found abundant literature published later where, in addition to the recommendations for the management of the 19 NPSLE syndromes defined by the ACR, additional recommendations are given for other neurological and/or psychiatric syndromes, conditions, and complications that have been associated to SLE in recent years. We review below the diagnostic and therapeutic management of the different entities.
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Lúpus Eritematoso Sistêmico , Vasculite Associada ao Lúpus do Sistema Nervoso Central , Humanos , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/epidemiologia , Vasculite Associada ao Lúpus do Sistema Nervoso Central/diagnóstico , Vasculite Associada ao Lúpus do Sistema Nervoso Central/epidemiologia , Vasculite Associada ao Lúpus do Sistema Nervoso Central/terapia , PrevalênciaRESUMO
Isolated extrapulmonary involvement in sarcoidosis is uncommon and reported in 5-9% of systemic sarcoidosis, this constitutes a clinical challenge due to its extensive differential diagnosis. Extrapulmonary sarcoidosis affecting more than three organs is rarely reported and there are scarce literature data published on diagnosis, clinical course and management in those cases. We hereby discuss a case of a 41-year-old female with systemic non-pulmonary sarcoidosis affecting lacrimal gland, peripheral lymph nodes, parotid gland and the liver.
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OBJECTIVES: To describe the methods of the Spanish Registry of patients with idiopathic inflammatory myopathy (IIM) (Myo-Spain), as well as its strengths and limitations. The main objective of the project is to analyse the evolution and clinical management of a cohort of patients with IIM. METHODS: Observational, longitudinal, ambispective and multicentre study of a cohort of patients with IIM seen in rheumatology units in Spain. All patients with a diagnosis of IMM will be included in the regular follow-up of the participating centres, regardless of age on initiation of the process. Incident cases will be all patients who at the beginning of the study have been diagnosed for less than 12 months and prevalent cases for more than 12 months. The registry will include data from the visit at baseline, one year and two years. Socio-demographic, clinical, analytical variables, complications, comorbidities, association with other rheumatic diseases, hospital admissions, mortality and treatments will be collected. In addition, indices, scales and questionnaires of activity, muscle involvement, damage, disability, and quality of life will be determined. The recruitment period will be 23 months. The purpose is to obtain a cohort of 400 patients with IMM. CONCLUSIONS: Myo-Spain registry provides the opportunity to develop a cohort of incident and prevalent patients with IMM in Spain. Myo-Spain will be able to assess in detail the clinical characteristics of the disease at different times. The comprehensive information collected during the visits is expected to provide a broad source of data for future analysis.
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BACKGROUND: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that affects multiple organs and systems. B cells have a critical role in the pathogenesis of SLE. Rituximab (RTX) is a drug composed of chimeric monoclonal antibodies against the CD20 protein, producing a depletion of B lymphocytes. OBJECTIVE: To analyze the effectiveness and safety of RTX in patients with SLE in clinical practice. METHODS: Collection of retrospective variables of the medical records of 20 patients with SLE treated with RTX in 2hospitals (Hospital de la Santa Creu i Sant Pau, and Hospital del Mar, in Barcelona, Spain). We evaluated demographic, clinical, serological and treatment variables. RESULTS: There was a statistically significant association in the following variables collected in the study before and after treatment: there was a decrease in the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) (P<.001), erythrocyte sedimentation rate (P=.017), use of glucocorticoids (P=.025) and IgM values (P=.031), as well as an increase in the C4 values (P=.014) after treatment with RTX. A patient with SLE, antiphospholipid syndrome, complex comorbidity and multiorgan lupus involvement died after developing a septic process, months after receiving a single treatment cycle with RTX. CONCLUSIONS: Although RTX currently has no official indication approved for SLE, our data suggest that it may be effective in reducing the activity of the disease and as a steroid-sparing agent, with an acceptable safety profile. However, larger follow-up periods with a greater number of patients are needed to solve the remaining doubts about the use of RTX in SLE.
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Fatores Imunológicos/uso terapêutico , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Rituximab/uso terapêutico , Adulto , Feminino , Humanos , Fatores Imunológicos/efeitos adversos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Rituximab/efeitos adversos , Resultado do TratamentoRESUMO
Systemic lupus erythematosus (SLE) is characterized by a wide variety of manifestations and a difficult disease control in some patients. We present the case of a 51-year-old woman who presented with a flare of SLE including arthritis and pleuropericarditis that responded to neither leflunomide, methotrexate, nor high doses of prednisone. After initiating treatment with belimumab, the patient experienced a quick and notorious improvement. She remains stable to this day while continuing belimumab therapy.
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Chikungunya virus infection (CHIKV) is associated with joint involvement in half of the cases. This can lead to erosive arthritis which, given the high intervariability of clinical and serological presentations, and the probable role of genetic conditioning in the severity and chronification of the condition, represents a great diagnostic and therapeutic challenge. There is an important lack of scientific evidence that would enable us to characterize the variability of the patient and choose the most appropriate approach.
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Artrite/virologia , Adulto , Febre de Chikungunya , Feminino , HumanosRESUMO
OBJECTIVES: Lupus nephritis (LN) requires renal replacement therapy in 10%-30% of patients. About 30% of these patients receive a kidney transplant. Belatacept is a second-generation, selective, T-cell co-stimulator blocker (inhibits cytotoxic, T-lymphocyte antigen 4, CTLA-4) used as an alternative to calcineurin inhibitors (CNI) for maintenance regimens after kidney transplantation. The pathogenic relevance of CTLA-4 inhibition and the favourable cardiovascular profile of belatacept make it an attractive therapeutic option in systemic lupus erythematosus (SLE). Intravenous administration of belatacept ensures therapeutic adherence. METHODS: This retrospective, single-centre study evaluates the outcomes of LN kidney transplant recipients treated with belatacept for reasons not related to SLE at the Columbia University Lupus and Renal Transplant Cohort. RESULTS: Belatacept was started in six patients on CNI regimens at 15.5±17.1 months following transplantation for LN. In five patients, creatinine levels stabilised 6 months after belatacept, one returned to haemodialysis due to CNI toxicity and pyelonephritis and one relisted for a kidney transplant following acute cellular rejection and cortical necrosis. Five patients are followed for extrarenal lupus; no extrarenal manifestations were documented in the other two patients. Data on SLE disease activity pre-belatacept and post-belatacept were available and scored in three patients using the SLE Disease Activity Index Glucocorticosteroid Index (SLEDAI-2KG), which accounts for clinical and laboratory manifestations, as well as steroid dose. Mean SLEDAI-2KG decreased from 13 to 7.6. CONCLUSION: Belatacept in LN kidney transplant recipients may decrease extrarenal manifestations, attenuate CNI toxicity and stabilise allograft function, providing a better alternative to CNI regimens. Furthermore, these data suggest that belatacept, although initiated for reasons not related to SLE, might have a beneficial effect in SLE.
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Systemic lupus erythematosus (SLE) is an autoimmune disease with a wide clinical expression, and musculoskeletal manifestations are the most frequent manifestations of the disease. Digestive manifestations, however, are less prevalent, appearing in only 2%-30% of the patients. Among these cases, the association of SLE with autoimmune hepatitis (AIH) is considered to be rare, with only a few cases documented in the medical literature. We present the only reported case to date of a patient diagnosed with SLE suffering from a flare with a simultaneous hepatic and muscular inflammatory involvement, both evidenced by biopsy.
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OBJECTIVE: To determine the prevalence of fatigue in our cohort as well as the factors to which it is associated, its relationship with demographic variables, vitamin D levels, treatment, systemic lupus erythematosus (SLE) symptoms and disease activity. METHODS: A cross-sectional study was carried out including 102 consecutive female patients with SLE (American College of Rheumatology 1997 criteria) who attended the Parc de Salut Mar between January 2012 and May 2014. Variables collected were: sociodemographic data, vitamin D supplementation, fatigue VAS, pharmacological treatment, main serological markers of SLE, and plasma levels of 25(OH)-vitD. The association between fatigue and the different variables was evaluated by the Spearman's Rho correlation coefficient for the continuous variables, the Mann-Whitney U test for the categorical and the Kruskal-Wallis test for the seasons of the year. RESULTS: The fatigue variable was evaluated through a fatigue VAS with a mean score of 52.84 (range 0-100), a median of 59 and a standard deviation of 29.86. A statistically significant relationship was found between fatigue and age, MHAQ, SLICC, summer and photosensitivity. As for the relationship between fatigue and vitamin D insufficiency (defined as 25-(OH)-vitD≤30 levels), the sample was divided into patients receiving vitamin D supplements (n=60) and patients without supplements (n=40), finding a significant relationship in that last group. CONCLUSIONS: A statistically significant association was found between the presence of fatigue and age, MHAQ, SLICC, photosensitivity, fibromyalgia and summer, and with vitamin D insufficiency in the group of patients without supplements (n=40).