To be or not to B27 positive: implications for the phenotypes of axial spondyloarthritis outcomes. Data from a large multiracial cohort from the Brazilian Registry of Spondyloarthritis.

BACKGROUND: There is a remarkable variability in the frequency of HLA-B27 positivity in patients with spondyloarthritis (SpA), which may be associated with different clinical presentations worldwide. However, there is a lack of data considering ethnicity and sex on the evaluation of the main clinical and prognostic outcomes in mixed-race populations. The aim of this study was to evaluate the frequency of HLA-B27 and its correlation with disease parameters in a large population of patients from the Brazilian Registry of Spondyloarthritis (RBE). METHODS: The RBE is a multicenter, observational, prospective cohort that enrolled patients with SpA from 46 centers representing all five geographic regions of Brazil. The inclusion criteria were as follow: (1) diagnosis of axSpA by an expert rheumatologist; (2) age ≥18 years; (3) classification according to ASAS axial. The following data were collected via a standardized protocol: demographic data, disease parameters and treatment historical. RESULTS: A total of 1096 patients were included, with 73.4% HLA-B27 positivity and a mean age of 44.4 (±13.2) years. Positive HLA-B27 was significantly associated with male sex, earlier age at disease onset and diagnosis, uveitis, and family history of SpA. Conversely, negative HLA-B27 was associated with psoriasis, higher peripheral involvement and disease activity, worse quality of life and mobility. CONCLUSIONS: Our data showed that HLA-B27 positivity was associated with a classic axSpA pattern quite similar to that of Caucasian axSpA patients around the world. Furthermore, its absence was associated with peripheral manifestations and worse outcomes, suggesting a relevant phenotypic difference in a highly miscegenated population.

Evaluation of obstetric outcomes in Brazilian pregnant women with Takayasu arteritis.

OBJECTIVE: Takayasu arteritis (TAK) is a rare chronic granulomatous vasculitis that affects large vessels and usually begins in women of childbearing age, so it is not uncommon for pregnancies to occur in these patients. However, there is limited information about these pregnancies, with reports of adverse maternal and obstetric outcomes. The objective of this study is to evaluate adverse maternal, fetal and neonatal events in pregnant patients with TA. METHODS: This is a cross-sectional study with retrospective data collection. We reviewed 22 pregnancies in 18 patients with TAK, according to the American College of Rheumatology criteria, that were followed up in a high-risk prenatal clinic specialized in systemic autoimmune diseases and thrombophilia (PrAT) at Hospital Universitário Pedro Ernesto, from 1998 to 2021. RESULTS: In twenty-two pregnancies, the mean age of patients was 28.09 years and the mean duration disease was 10.9 years. Of the 18 patients with TAK studied, only one had the diagnosis during pregnancy and had active disease. All other patients had a previous diagnosis of TAK and only 3 had disease activity during pregnancy. Twelve patients (66.6%) had previous systemic arterial hypertension and eleven (61.1%) had renal involvement. Among maternal complications, eight patients (36.3%) developed preeclampsia and six (27.2%) had uncontrolled blood pressure without proteinuria, while 10 (45%) had puerperal complications. Four (18.1%) births were premature, all due to severe preeclampsia and eight newborns (34.7%) were small for gestational age. When all maternal and fetal/neonatal outcomes included in this study were considered, only 6 (27.2%) pregnancies were uneventful. CONCLUSION: Although there were no maternal deaths or pregnancy losses in this study, the number of adverse events was considerably high. Hypertensive disorders and small for gestational age newborns were more common than general population, while the number of patients with active disease was low. These findings suggest that pregnancies in patients with TAK still have several complications and a high-risk prenatal care and delivery are necessary for these patients.

Evaluation of obstetric outcomes in Brazilian pregnant women with Takayasu arteritis

Abstract Objective Takayasu arteritis (TAK) is a rare chronic granulomatous vasculitis that affects large vessels and usually begins in women of childbearing age, so it is not uncommon for pregnancies to occur in these patients. However, there is limited information about these pregnancies, with reports of adverse maternal and obstetric outcomes. The objective of this study is to evaluate adverse maternal, fetal and neonatal events in pregnant patients with TA. Methods This is a cross-sectional study with retrospective data collection. We reviewed 22 pregnancies in 18 patients with TAK, according to the American College of Rheumatology criteria, that were followed up in a high-risk prenatal clinic specialized in systemic autoimmune diseases and thrombophilia (PrAT) at Hospital Universitário Pedro Ernesto, from 1998 to 2021. Results In twenty-two pregnancies, the mean age of patients was 28.09 years and the mean duration disease was 10.9 years. Of the 18 patients with TAK studied, only one had the diagnosis during pregnancy and had active disease. All other patients had a previous diagnosis of TAK and only 3 had disease activity during pregnancy. Twelve patients (66.6%) had previous systemic arterial hypertension and eleven (61.1%) had renal involvement. Among maternal complications, eight patients (36.3%) developed preeclampsia and six (27.2%) had uncontrolled blood pressure without proteinuria, while 10 (45%) had puerperal complications. Four (18.1%) births were premature, all due to severe preeclampsia and eight newborns (34.7%) were small for gestational age. When all maternal and fetal/neonatal outcomes included in this study were considered, only 6 (27.2%) pregnancies were uneventful. Conclusion Although there were no maternal deaths or pregnancy losses in this study, the number of adverse events was considerably high. Hypertensive disorders and small for gestational age newborns were more common than general population, while the number of patients with active disease was low. These findings suggest that pregnancies in patients with TAK still have several complications and a high-risk prenatal care and delivery are necessary for these patients.

Epidemiologic Features of Systemic Vasculitides in the Southeast Region of Brazil: Hospital-Based Survey.

BACKGROUND/OBJECTIVE: The epidemiology of vasculitis is variable in different geographic areas, and this issue has not been approached in Brazil yet. The objective of this study was to assess the frequency of vasculitis in specialized centers in Brazil. METHODS: This cross-sectional study was performed in 9 vasculitis outpatient clinics from 6 different states mainly from the Southeast and the Northeast regions of Brazil between 2015 and 2017. Diagnosis and/or classification criteria for Behçet disease (BD), Takayasu arteritis (TA), giant cell arteritis (GCA), polyarteritis nodosa (PAN), granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), and cryoglobulinemic vasculitis (CryoVas) were used to include patients with at least 6 months of follow-up in this hospital-based survey. RESULTS: A total of 1233 patients with systemic vasculitis were included from the Southeast region. Behçet disease was the most frequent vasculitis (35.0%) followed by TA (26.4%), GPA (16.2%), PAN (5.8%), GCA (5.8%), EGPA (4.3%), MPA (3.4%), and CryoVas (3.0%). Up to 7.8% of vasculitis patients had a juvenile onset, and the frequency of vasculitides found in children and adolescents was as follows: TA (52.6%), BD (24.7%), GPA (12.4%), and PAN (10.3%). No cases of EGPA, MPA, and CryoVas were diagnosed before the age of 18 years. As a comparator, 103 vasculitis patients were included in the Northeast of Brazil where TA was found in 36.9% and BD in 31.1% of vasculitis cases. No GCA cases were found in the Northeast part of Brazil. CONCLUSIONS: Similar to the epidemiology of vasculitis in Asia, BD and TA are the most frequent vasculitis in Southeastern Brazilian referral centers.

Recommendations of the Brazilian Society of Rheumatology for the induction therapy of ANCA-associated vasculitis.

The purpose of these recommendations is to guide the appropriate induction treatment of antineutrophil cytoplasmic antibody-associated vasculitis (AAV) patients with active disease. The recommendations proposed by the Vasculopathies Committee of the Brazilian Society Rheumatology for induction therapy of AAV, including granulomatosis with polyangiitis, microscopic polyangiitis and renal-limited vasculitis, were based on systematic literature review and expert opinion. Literature review was performed using Medline (PubMed), EMBASE and Cochrane database to retrieve articles until October 2016. PRISMA guidelines were used for the systematic review and articles were assessed according to the Oxford levels of evidence. Sixteen recommendations were made regarding different aspects of induction therapy for AAV. The purpose of these recommendations is to serve as a guide for therapeutic decisions by health care professionals in the management of AAV patients presenting active disease.

Ocular involvement in patients with spondyloarthritis.

Ocular inflammatory diseases can present as isolated conditions but also as part of systemic inflammatory diseases. Anterior uveitis is closely related to SpA and shares the common genetic background of HLA-B27. Other ocular manifestations, such as episcleritis and scleritis, may also occur, although less frequently. Therefore, ocular involvement has been included as one of the important clinical features of SpA in the recently published classification criteria for axial and peripheral disease. However, there are a wide variety of aetiologies for ocular diseases and this must be considered in assessment of SpA.

Recommendations of the Brazilian Society of Rheumatology for the induction therapy of ANCA-associated vasculitis / Recomendações da Sociedade Brasileira de Reumatologia para a terapia de indução para vasculite associada a ANCA

Abstract The purpose of these recommendations is to guide the appropriate induction treatment of antineutrophil cytoplasmic antibody-associated vasculitis (AAV) patients with active disease. The recommendations proposed by the Vasculopathies Committee of the Brazilian Society Rheumatology for induction therapy of AAV, including granulomatosis with polyangiitis, microscopic polyangiitis and renal-limited vasculitis, were based on systematic literature review and expert opinion. Literature review was performed using Medline (PubMed), EMBASE and Cochrane database to retrieve articles until October 2016. PRISMA guidelines were used for the systematic review and articles were assessed according to the Oxford levels of evidence. Sixteen recommendations were made regarding different aspects of induction therapy for AAV. The purpose of these recommendations is to serve as a guide for therapeutic decisions by health care professionals in the management of AAV patients presenting active disease.

Resumo O objetivo destas recomendações é orientar o tratamento apropriado de indução em pacientes com vasculite associada a anticorpos anticitoplasma de neutrófilos (VAA) ativa. As recomendações propostas pelo Comitê de Vasculopatias da Sociedade Brasileira de Reumatologia para a terapia de indução para vasculites associadas aos anticorpos anticitoplasma de neutrófilos (VAA), inclusive granulomatose com poliangiite, poliangiite microscópica e vasculite limitada ao rim, foram baseadas em uma revisão sistemática da literatura e na opinião de especialistas. A revisão da literatura foi feita com as bases de dados Medline (PubMed), Embase e Cochrane para consultar artigos até outubro de 2016. As diretrizes Prisma (Preferred Reporting Items for Systematic Reviews and Meta-Analyses - Principais itens para reportar revisões sistemáticas e metanálises) foram usadas para a revisão sistemática e os artigos foram avaliados de acordo com os níveis de evidência Oxford. Dezesseis recomendações foram feitas em relação a diferentes aspectos da terapia de indução para VAA. O objetivo dessas recomendações é servir como um guia para decisões terapêuticas por profissionais da saúde no tratamento de pacientes com VAA que apresentem a doença ativa.

Abordagem terapêutica das vasculites pulmonares / Therapeutic approach to pulmonary vasculitis

Muitas vasculites sistêmicas acometem o trato respiratório. O acometimento mais frequente é o do parênquima pulmonar pelas vasculites de pequenos vasos ANCA associadas (antineutrophil cytoplasmic antibodies), que incluem a granulomatose com poliangite (GPA, antiga granulomatose de Wegener), a poliangite microscópica (MPA), granulomatose eosinofílica com poliangite (EGPA, antiga doença de Churg-Strauss). Este artigo propõe-se a revisar o tratamento dessas doenças de acordo com recomendações da liga europeia de combate ao reumatismo (EULAR), com base no estádio e na atividade de doença, incluindo as terapia usadas na indução e na manutenção de remissão, assim como nos casos refratários ao tratamento convencional

There are many vasculitis that affect the respiratory tract. The pulmonary parenchyma is the most frequently involved and occurs mainly in ANCA associated small vessel vasculitis (antineutrophil cytoplasmic antibodies), which include granulomatosis with polyangitis (GPA, formely Wegener's granulomatosis), microscopic polyangitis (MPA), eosinophilic granulomatosis with polyangitis (EGPA formely Churg-Strauss Syndrome). This article reviews the treatment of them, according to EULAR (European League Against Rheumatism) recommendations, which are based on stage and severity of disease, including induction and maintance therapy, and therapy of refractory disease

Classificação e clínica das vasculites pulmonares / Clinical classification of pulmonary vasculitis

As vasculites sistêmicas são doenças raras e heterogêneas que acometem diferentes sistemas. A gravidade da manifestação clínica depende do tamanho, local e extensão do comprometimento do leito vascular. O reconhecimento das manifestações clínicas por diferentes especialidades médicas permite o diagnóstico e o tratamento precoce, interferindo decisivamente no prognóstico. Muitas dessas doenças acometem o trato respiratório e o pneumologista é, com frequência, o especialista envolvido na propedêutica inicial. É importante conhecer as formas de classificação desenvolvidas na tentativa de categorizar as vasculites. O presente artigo propõe-se a rever tais classificações, assim como as doenças que acometem o trato respiratório e os possíveis diagnósticos diferenciais que costuam mimetizar as vasculites. Destacamos que o acometimento pulmonar ocorre principalmente nas vasculites de pequenos vasos, associados ao ANCA (antineutrophil cytoplasmic antibodies), que incluem a granulomatose com poliangite (GPA, antiga granulomatose de Wegener), a poliangite microscópica (MPA), granulomatose eosinofílica com poliangite (EGPA, antiga doença de Churg-Strauss). A respeito dos critérios classificatórios do American College of Rheumatology (ACR), reforçamos que servem para nomear e classificar a vasculite previamente documentada, mas não incluem o exame de ANCA e apresentam restrições em separar pacientes com GPA e MPA

Systemic vasculitis are rare and heterogenous diseases that affect different systems. The severity of clinical manifestation depends on the size, location and distribution of vessel involvement. The prompt recognition of the clinical manifestations allows early diagnosis and treatment, improving the prognosis. There are many vasculitis that affect the respiratory tract, therefore the pulmonologist is often a specialist involved in the initial workup. It is important to know the forms of classification developed in an attempt to categorize vasculitis. This article proposes to revise such classification, as well as diseases affecting the respiratory tract and the possible diferential diagnoses that often mimic vasculitis. We emphasize that the pulmonary involvement occurs mainly in ANCA-associated smal vessel vasculitis (antineutrophil cytoplasmic antibodies), which include granumatosis with polyangitis (GPA, formely Wegener's granulomatosis), microscopic polyangitis (MPA), eosinophilic granulomatosis with polyangitis (EGPA formely Churg-Strauss Syndrome). Regarding the classification criteria of the American College of Rheumatology (ACR), it is important to reinforce its use in naming and classifying vasculitis after rule out infectious and neoplasic diseases, but remember that it does not include the ANCA exam and has its limits in separating patients with GPA and MPA A