To analyze the clinical and epidemiologic characteristics of juvenile-onset spondyloarthritis (SpA) (< 16 years) and compare them with a group of adult-onset (≥ 16 years) SpA patients. Prospective, observational and multicentric cohort with 1,424 patients with the diagnosis of SpA according to the European Spondyloarthropathy Study Group (ESSG) submitted to a common protocol of investigation and recruited in 29 reference centers participants of the Brazilian Registry of Spondyloarthritis (RBE – Registro Brasileiro de Espondiloartrites). Patients were divided in two groups: age at onset < 16 years (JOSpA group) and age at onset ≥ 16 years (AOSpA group). Among the 1,424 patients, 235 presented disease onset before 16 years (16.5%). The clinical and epidemiologic variables associated with JOSpA were male gender (p < 0.001), lower limb arthritis (p = 0.001), enthesitis (p = 0.008), anterior uveitis (p = 0.041) and positive HLA-B27 (p = 0.017), associated with lower scores of disease activity (Bath Ankylosing Spondylitis Disease Activity Index – BASDAI; p = 0.007) and functionality (Bath Ankylosing Spondylitis Functional Index – BASFI; p = 0.036). Cutaneous psoriasis (p < 0.001), inflammatory bowel disease (p = 0.023), dactylitis (p = 0.024) and nail involvement (p = 0.004) were more frequent in patients with adult-onset SpA. Patients with JOSpA in this large Brazilian cohort were characterized predominantly by male gender, peripheral involvement (arthritis and enthesitis), positive HLA-B27 and lower disease scores. Analisar as características clínicas e epidemiológicas da espondiloartrite (EspA) de início juvenil (< 16 anos) e compará-las com um grupo de pacientes com EspA de início na vida adulta (≥ 16 anos). Coorte prospectiva, observacional e multicêntrica com 1.424 pacientes com diagnóstico de EspA de acordo com o European Spondyloarthropathy Study Group (ESSG) submetidos a um protocolo comum de investigação e recrutados em 29 centros de referência participantes do Registro Brasileiro de Espondiloartrites (RBE). Os pacientes foram divididos em dois grupos: idade no início < 16 anos (grupo EspAiJ) e idade no início ≥ 16 anos. Entre os 1.424 pacientes, 235 manifestaram o início da doença antes dos 16 anos (16,5%). As variáveis clínicas e epidemiológicas associadas com a EspAiJ foram: gênero masculino (p < 0,001), artrite em membro inferior (p = 0,001), entesite (p = 0,008), uveíte anterior (p = 0,041) e HLA-B27 positivo (p = 0,017), em associação com escores mais baixos de atividade da doença (Bath Ankylosing Spondylitis Disease Activity Index – BASDAI; p = 0,007) e de capacidade funcional (Bath Ankylosing Spondylitis Functional Index – BASFI; p = 0,036). A psoríase cutânea (p < 0,001), a doença intestinal inflamatória (p = 0,023), a dactilite (p = 0,024) e o envolvimento ungueal (p = 0,004) foram mais frequentes em pacientes com EspA de início na vida adulta. Nessa grande coorte brasileira, os pacientes com EspAiJ se caracterizavam predominantemente pelo gênero masculino, envolvimento periférico (artrite e entesite), HLA-B27 positivo e escores de doença mais baixos.
To assess the incidence of tuberculosis and to screen for latent tuberculosis infection among Brazilians with rheumatoid arthritis using biologics in clinical practice. This cohort study used data from the Brazilian Registry of Biological Therapies in Rheumatic Diseases (Registro Brasileiro de Monitoração de Terapias Biológicas – BiobadaBrasil), from 01/2009 to 05/2013, encompassing 1552 treatments, including 415 with only synthetic disease-modifying anti-rheumatic drugs, 942 synthetic DMARDs combined with anti-tumor necrosis factor (etanercept, infliximab, adalimumab) and 195 synthetic DMARDs combined with other biologics (abatacept, rituximab and tocilizumab). The occurrence of tuberculosis and the drug exposure time were assessed, and screening for tuberculosis was performed. Statistical analysis: Unpaired t-test and Fisher's two-tailed test; p < 0.05. The exposure times were 981 patient-years in the controls, 1744 patient-years in the anti-TNF group (adalimumab = 676, infliximab = 547 and etanercept = 521 patient-years) and 336 patient-years in the other biologics group. The incidence rates of tuberculosis were 1.01/1000 patient-years in the controls and 2.87 patient-years among anti-TNF users (adalimumab = 4.43/1000 patient-years; etanercept = 1.92/1000 patient-years and infliximab = 1.82/1000 patient-years). No cases of tuberculosis occurred in the other biologics group. The mean drug exposure time until the occurrence of tuberculosis was 27(11) months for the anti-TNF group. The incidence of tuberculosis was higher among users of synthetic DMARDs and anti-TNF than among users of synthetic DMARDs and synthetic DMARDs and non-anti-TNF biologics and also occurred later, suggesting infection during treatment and no screening failure. Avaliar incidência de tuberculose e triagem para tuberculose latente em brasileiros com artrite reumatoide em uso de agentes biológicos na prática clinica. Estudo de coorte com dados do Registro Brasileiro de Monitoração de Terapias Biológicas (BiobadaBrasil), de 01/2009 a 05/2013, abrangeu 1.552 tratamentos, 415 somente com drogas modificadoras do curso da doença (MMCDs) sintéticas, 942 MMCDs sintéticas em associação com anti-TNF (etanercepte, infliximabe, adalimumabe) e 195 MMCDs sintéticas em associação com outros biológicos (abatacepte, rituximabe e tocilizumabe). Avaliaram-se ocorrência de tuberculose, tempo de exposição às drogas e triagem para tuberculose. Análise estatística: teste t não pareado e teste de Fisher bicaudal; p < 0,05. O tempo de exposição dos controles foi de 981 pacientes-ano, do grupo de anti-TNF foi de 1.744 pacientes-ano (adalimumabe = 676, infliximabe = 547 e etanercepte = 521 pacientes-ano) e o de outros biológicos de 336 pacientes-ano. A incidência de tuberculose foi de 1,01/1.000 pacientes-ano nos controles e de 2,87 pacientes-ano nos usuários de anti-TNF (adalimumabe = 4,43/1.000 pacientes-ano; etanercepte = 1,92/1.000 pacientes-ano e infliximabe = 1,82/1.000 pacientes-ano). Não houve casos de tuberculose no grupo de outros biológicos. O tempo médio de exposição até a ocorrência de tuberculose foi de 27(11) meses para o grupo anti-TNF. A incidência de tuberculose foi maior nos usuários de MMCDs sintéticas e anti-TNF do que nos usuáris de MMCDs sintéticas e de MMCDs sintéticas e biológicos não anti-TNF, e também mais tardia, sugerindo infecção durante o tratamento, e não falha na triagem.
Inflammatory bowel diseases (Crohn's disease and ulcerative rectocolitis) have extraint- estinal manifestations 25% of the patients, with the most common one being the entero- pathic arthritis. Prospective, observational, multicenter study with patients from 29 reference cen- ters participating in the Brazilian Registry of Spondyloarthritis (RBE), which incorporates the RESPONDIA (Ibero-American Registry of Spondyloarthritis) group. Demographic and clinical data were collected from 1472 patients and standardized questionnaires for the as- sessment of axial mobility, quality of life, enthesitic involvement, disease activity and func- tional capacity were applied. Laboratory and radiographic examinations were performed. The aim of this study is to compare the clinical, epidemiological, genetic, imaging, treat- ment and prognosis characteristics of patients with enteropathic arthritis with other types of spondyloarthritis in a large Brazilian cohort. A total of 3.2% of patients were classified as having enteroarthritis, 2.5% had spon- dylitis and 0.7%, arthritis (peripheral predominance). The subgroup of individuals with en- teroarthritis had a higher prevalence in women (P < 0.001), lower incidence of inflammatory axial pain (P < 0.001) and enthesitis (P = 0.004). HLA-B27 was less frequent in the group with enteroarthritis (P = 0.001), even when considering only those with the pure axial form. There was a lower prevalence of radiographic sacroiliitis (P = 0.009) and lower radiographic score (BASRI) (P = 0.006) when compared to patients with other types of spondyloarthritis. They also used more corticosteroids (P < 0.001) and sulfasalazine (P < 0.001) and less non- steroidal anti-inflammatory drugs (P < 0.001) and methotrexate (P = 0.001). There were differences between patients with enteroarthritis and other types of spondyloarthritis, especially higher prevalence of females, lower frequency of HLA-B27, associated with less severe axial involvement. As doenças inflamatórias intestinais (doença de Crohn e retocolite ulcerativa) apresentam manifestações extraintestinais em um quarto dos pacientes, sendo a mais comum a artrite enteropática. Estudo prospectivo, observacional e multicêntrico, realizado com pacientes de 29 centros de referência participantes do Registro Brasileiro de Espondiloartrites (RBE), que se incorpora ao grupo RESPONDIA (Registro Ibero-americano de Espondiloartrites). Dados demográficos e clínicos de 1472 pacientes foram colhidos, e aplicaram-se questionários pa- dronizados de avaliação de mobilidade axial, de qualidade de vida, de envolvimento entesí- tico, de atividade de doença e de capacidade funcional. Exames laboratoriais e radiográficos foram realizados. Objetivamos, neste presente artigo, comparar as características clínicas, epidemiológicas, genéticas, imagenológicas, de tratamento e prognóstico de enteroartríti- cos com os outros espondiloartríticos nesta grande coorte brasileira. Foram classificados como enteroartrite 3,2% dos pacientes, sendo que 2,5% ti- nham espondilite e 0,7%, artrite (predomínio periférico). O subgrupo de indivíduos com en- teroartrite apresentava maior prevalência de mulheres (P < 0,001), menor incidência de dor axial inflamatória (P < 0,001) e de entesite (P = 0,004). O HLA-B27 foi menos frequente no grupo de enteroartríticos (P = 0,001), mesmo se considerado apenas aqueles com a forma axial pura. Houve menor prevalência de sacroiliíte radiológica (P = 0,009) e também menor escore radiográfico (BASRI) (P = 0,006) quando comparado aos pacientes com as demais es- pondiloartrites. Também fizeram mais uso de corticosteroides (P < 0,001) e sulfassalasina (P < 0,001) e menor uso de anti-inflamatórios não hormonais (P < 0,001) e metotrexato (P = 0,001). Foram encontradas diferenças entre as enteroartrites e as demais espondiloar- trites, principalmente maior prevalência do sexo feminino, menor frequência do HLA-B27, associados a uma menor gravidade do acometimento axial.
To analyze the results of the application of the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) in a large series of Brazilian patients with the diagnosis of SpA and establish its correlations with specific variables into the group.A common protocol of investigation was prospectively applied to 1492 Brazilian patients classified as SpA according to the European Spondyoarthropathies Study Group (ESSG), attended at 29 referral centers of Rheumatology in Brazil. Clinical and demographic variables, and disease indices (BASDAI, Basfi, Basri, Mases, ASQol) were applicated. The total values of BASDAI were compared to the presence of the different variables.The mean score of BASDAI was 4.20 ± 2.38. The mean scores of BASDAI were higher in patients with the combined (axial + peripheral + entheseal) (4.54 ± 2.38) clinical presentation, compared to the pure axial (3.78 ± 2.27) or pure peripheral (4.00 ± 2.38) clinical presentations (p<0.001). BASDAI also presented higher scores associated with the female gender (p<0.001) and patients who did not practice exercises (p < 0.001). Regarding the axial component, higher values of BASDAI were significantly associated with inflammatory low back pain (p<0.049), alternating buttock pain (p<0.001), cervical pain (p<0.001) and hip involvement (p<0.001). There was also statistical association between BASDAI scores and the peripheral involvement, related to the lower (p=0.004) and upper limbs (p=0.025). The presence of enthesitis was also associated to higher scores of BASDAI (p=0.040). Positive HLA-B27 and the presence of cutaneous psoriasis, inflammatory bowel disease, uveitis and urethritis were not correlated with the mean scores of BASDAI. Lower scores of BASDAI were associated with the use of biologic agents (p<0.001).In this heterogeneous Brazilian series of SpA patients, BASDAI was able to demonstrate "disease activity" in patients with axial as well as peripheral disease.
Most reports on serious infections (SI) in rheumatoid arthritis (RA) patients treated with biological disease-modifying antirheumatic drugs (bDMARDs) are from the USA and Western Europe. Data from other regions are largely missing. We report data from South American countries with different backgrounds and health-care systems but similar registries. We merged 2010–2016 data from two registries, BIOBADABRASIL (Brazil) and BIOBADASAR (Argentina), which share the same protocol, online platform and data monitoring process. Patients with active RA were included when they began the first bDMARD or a conventional synthetic DMARD (csDMARD, control group). The SI incidence rate (IR) per 1000 patient/years and adjusted IR ratio (aIRR) were estimated for bDMARDs and csDMARDs. Data were analysed for 3717 RA patients with an exposure of 13,380 patient/years. The 2591 patients treated with bDMARDs (64% tumour necrosis factor-α inhibitors (TNFi)) had a follow-up of 9300 years, and the 1126 treated with csDMARDs had an exposure of 4081 patient/years. The SI IR was 30.54 (CI 27.18–34.30) for all bDMARDs and 5.15 (CI 3.36–7.89) for csDMARDs. The aIRR between the two groups was 2.03 ([1.05, 3.9] p = 0.034) for the first 6 months of treatment but subsequently increased to 8.26 ([4.32, 15.76] p < 0.001). The SI IR for bDMARDs decreased over time in both registries, dropping from 36.59 (28.41–47.12) in 2012 to 7.27 (4.79–11.05) in 2016. While SI remains a major concern in South American patients with RA treated with bDMARDs, a favourable trend toward a reduction was observed in the last years.
Abstract Spondyloarthritis (SpA) is a group of chronic inflammatory systemic diseases characterized by axial and/or peripheral joints inflammation, as well as extra-articular manifestations. Over some decades, nonsteroidal anti-inflammatory drugs (NSAIDs) have been the basis for the pharmacological treatment of patients with axial spondyloarthritis (axSpA). However, the emergence of the immunobiologic agents brought up the discussion about the role of NSAIDs in the management of these patients. The objective of this guideline is to provide recommendations for the use of NSAIDs for the treatment of axSpA. A panel of experts from the Brazilian Society of Rheumatology conducted a systematic review and meta-analysis of randomized clinical trials for 15 predefined questions. The Grading of Recommendations, Assessment, Development and Evaluation methodology to assess the quality of evidence and formulate recommendations were used, and at least 70% agreement of the voting panel was needed. Fourteen recommendations for the use of NSAIDs in the treatment of patients with axSpA were elaborated. The purpose of these recommendations is to support clinicians’ decision making, without taking out his/her autonomy when prescribing for an individual patient.
Inflammatory myositis is a heterogeneous group of diseases that produce muscle damage, a large number of these diseases do not respond to conventional treatment, requiring genetic studies.A variant of gem neb(nebulim, omim*16 1650), previously described in the medical literature, was reported in a bank of variants associated with Nemalinic Myopathy, a disease that affects 4% in adulthood, characterized by weakness of the Proximal and distal muscles, dysphagia, cervical ptosis, with normal or slightly elevated CPK and myopathic changes on electroneuromyography, thus diagnosed whit biopsy and modified Gomori trichromatic stain, to observe nemaline rods in muscle fibers, treatment with steroids and immunoglobulin with the improvement of 1/3 of patients and the most successful treatment was autologous peripheral blood stem cell transplantation 86% effective. CASE REPORTMale patient, 31 years old, white, with progressive weakness for 5 years, thrombosis of the left lower limb, started Daflon, AAS, and Xarelto for 5 months.Afterward, he is referred to a rheumatologist with a hypothesis of polymyositis.On physical examination, vital signs were unchanged, cardiovascular, respiratory, and gastrointestinal were normal, skeletal muscle with weakness in the deltoids, psoas and quadriceps.High levels of LDH, CK and Myoglobin.Electroneuromyography with axonal damage and small motor amplitude compatible with myopathy.He was treated with prednisone up to 100mg and MTX up to 20 mg, showing an apparent improvement in strength.Patients with irregular treatment lost to follow-up for 6 months.When he returned, his symptoms worsened, including dysphagia and motor ventilator disorder.Muscle biopsy shows a nonspecific and insufficient diagnosis for the characterization of myopathy.Started immunoglobulin 40 g for 5 days/month, without good response, the respiratory condition worsened with restrictive respiratory spirometry, methylprednisolone, pulse was started, evolved with palpebral ptosis, and abdomen CT scan and pelvic MRI showed edema, fatty infiltration and changes in muscle density.It exhibits negative results in genetic dystrophy research in Gene Duchene or Beker and Pompe research; in the sequencing of the complete exome, the variant associated with Nemalinic Myopathy was identified, and the patient died with severe respiratory restrictive condition after 5 years from the onset of symptoms. CONCLUSIONNemalic Myopathy is a very rare and still unknown disease, in the literature, there are 66 cases of meta-analysis from 1966 to 2016, with a diagnosis of 10 cases documented in recent years.
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