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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 Behcet 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. Behcet 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.

ObjectivesTo evaluate risk factors associated with unfavourable outcomes: emergency care, hospitalisation, admission to intensive care unit (ICU), mechanical ventilation and death in patients with immune-mediated rheumatic disease (IMRD) and COVID-19.MethodsAnalysis of the first 8 weeks of observational multicentre prospective cohort study (ReumaCoV Brasil register). Patients with IMRD and COVID-19 according to the Ministry of Health criteria were classified as eligible for the study.Results334 participants were enrolled, a majority of them women, with a median age of 45 years; systemic lupus erythematosus (32.9%) was the most frequent IMRD. Emergency care was required in 160 patients, 33.0% were hospitalised, 15.0% were admitted to the ICU and 10.5% underwent mechanical ventilation; 28 patients (8.4%) died. In the multivariate adjustment model for emergency care, diabetes (prevalence ratio, PR 1.38; 95% CI 1.11 to 1.73; p=0.004), kidney disease (PR 1.36; 95% CI 1.05 to 1.77; p=0.020), oral glucocorticoids (GC) (PR 1.49; 95% CI 1.21 to 1.85; p50 years (PR 1.89; 95% CI 1.26 to 2.85; p=0.002), no use of tumour necrosis factor inhibitor (TNFi) (PR 2.51;95% CI 1.16 to 5.45; p=0.004) and methylprednisolone pulse therapy (PR 2.50; 95% CI 1.59 to 3.92; pConclusionsAge >50 years and immunosuppression with GC and cyclophosphamide were associated with unfavourable outcomes of COVID-19. Treatment with TNFi may have been protective, perhaps leading to the COVID-19 inflammatory process.

Background:The role of chronic use of hydroxychloroquine (HCQ) in rheumatic disease (RD) patients during the SARS-CoV-2 pandemic is still subject of discussion.Objectives:To compare the occurrence of COVID-19 and its outcomes between RD patients on HCQ use with individuals from the same household not taking the drug during community viral transmission in an observational prospective multicenter study in Brazil.Methods:Participants were enrolled and monitored through 24-week (From March 29th to Sep 30th, 2020) regularly scheduled phone calls performed by trained medical professionals. Epidemiological and demographic data, as well as RD disease activity status and current treatment data, specific information about COVID-19, hospitalization, need for intensive care, and death was recorded in both groups and stored in the Research Electronic Data Capture (REDCap) database. COVID-19 was defined according to the Brazilian Ministry of Health (BMH) criteria. The statistical analysis was performed using IBM-SPSS v.20.0 software. Group comparisons were made using the Man-Whitney, Chi-Square and Fisher Exact Test, as well as multivariate regression models adjusted to confounders. Survival curves were performed using Kaplan-Meier analysis.Results:A total of 10,427 participants mean age (SD) of 44.04 (14.98) years were enrolled, including 6004 (57.6%) rheumatic disease patients, of whom 70.8% had systemic lupus erythematosus (SLE), 6.7% rheumatoid arthritis (RA), 4% primary Sjögren’s syndrome (pSS), 1.8% mixed connective tissue disease (DMTC), 1% systemic sclerosis (SSc) and others (15.9), including overlap syndromes. In total, 1,132 (10.8%) participants fulfilled criteria for COVID-19, being 6.7% RD patients and 4.1% controls (p=0.002). A recent influenza vaccination had a protective role (p=65 years (OR=0.20; 95%CI 0.11-0.34) was negatively associated. Regarding RD, the risk factors associated with COVID-19 diagnosys were SLE (OR= 2.37; 95%CI 1.92-293), SSc (OR=2.25; 95%CI 1.05-4.83) and rituximab use (OR=1.92; 95%CI 1.13-3.26). In addition, age >=65 years (OR=5.47; 95%CI 1.7-19.4) and heart disease (OR=2.60; 95%CI 1.06-6.38) were associated with hospitalization. Seven female RD patients died, six with SLE and one with pSS, and the presence of two or more comorbidities were associated with higher mortality rate.Conclusion:Chronic HCQ use did not prevent COVID-19 in RD compared to their household cohabitants. Health care profession, presence of comorbidities LES, SSc and rituximab were identified as main risk factors for COVID-19 and aging and heart disease as higher risk for hospitalization. Our data suggest these outcomes could be considered to manage them in clinical practice.Table 1.Frequency and severity of COVID-19 in patients with rheumatic diseases on chronic use of hydroxychloroquine compared to their household controlsCOVID-19 outcomesTotal(%)GroupsPPatients(%)Controls (%)DiagnosisNo9256 (89.1)5300 (88.3)3956 (90.2)0.002Yes1132 (10.9)704 (11.7)428 (9.8)SeverityMild1059 (93.6)662 (94.0)397 (92.8)0.391Moderate52 (4.6)32 (4.5)20 (4.7)Severe21 (1.9)10 (1.4)11 (2.6)HCQ: hydroxychloroquine.Moderate and severe COVID-19 included the need for any of the following: hospitalization, intensive care, mechanical ventilation or death.Acknowledgements:To the Brazilian Society of Rheumatology for technical support and rapid nationwide mobilization.To all the 395 interviewers (medical students and physicians) who collaborated in the study and the participantsTo CNPq (Number 403442/2020-6)Disclosure of Interests:None declared

Resumo O objetivo destas recomendacoes e orientar o tratamento apropriado de inducao em pacientes com vasculite associada a anticorpos anticitoplasma de neutrofilos (VAA) ativa. As recomendacoes propostas pelo Comite de Vasculopatias da Sociedade Brasileira de Reumatologia para a terapia de inducao para vasculites associadas aos anticorpos anticitoplasma de neutrofilos (VAA), inclusive granulomatose com poliangiite, poliangiite microscopica e vasculite limitada ao rim, foram baseadas em uma revisao sistematica da literatura e na opiniao de especialistas. A revisao da literatura foi feita com as bases de dados Medline (PubMed), Embase e Cochrane para consultar artigos ate outubro de 2016. As diretrizes Prisma ( Preferred Reporting Items for Systematic Reviews and Meta‐Analyses – Principais itens para reportar revisoes sistematicas e metanalises) foram usadas para a revisao sistematica e os artigos foram avaliados de acordo com os niveis de evidencia Oxford. Dezesseis recomendacoes foram feitas em relacao a diferentes aspectos da terapia de inducao para VAA. O objetivo dessas recomendacoes e servir como um guia para decisoes terapeuticas por profissionais da saude no tratamento de pacientes com VAA que apresentem a doenca ativa.

Background:Mental health was widely affected during the new coronavirus pandemic. In addition, some measures adopted by most countries in order to contain the virus spread, such as isolation and social distancing, leading to the interruption of routine activities, including partial or complete interruption of face-to-face classes may be associated with increased stress, depression and anxiety among undergraduate medical students (1). From March to September, 2020, the Brazilian Society of Rheumatology carried out the Mario Pinotti II Project (MPII), a prospective, multicenter, observational cohort study designed to monitor the COVID-19 in patients with rheumatic disease on hydroxychloroquine, using periodic telephone calls performed by undergraduate medical students (2).Objectives:To compare the mental health status of medical students who were participating from the MPII with theirs colleagues not involved in this project.Methods:A web-based survey via google forms platform was developed by a panel composed of undergraduate medical students, rheumatologists, medical school professors, and a psychology professor. It included details on demographic and life habits data and domains regarding depression, anxiety and stress, using the DASS-21 (Depression, Anxiety & Stress Scale), Brazilian version. Data collection occurred from July 20th to August 31st, 2020. Statistical analysis was performed using the SPSS version 20.0. Univariate and multivariate linear regression analysis were performed to verify associations with the DASS-21, defined as dependent variable. A p-value < 0.05 was deemed as significant. This study was approved by the Institutional Research Ethics Committee.Results:A total of 684 undergraduate medical students were included in this study, of whom 228 as MPII volunteers (VG) and 456 as control group (CG). Median age was 23 years (IQ 21-24) and the CG was older than the VG (pConclusion:Several aspects may be involved with mental health, including increased emotional maturity, gender and sleep pattern. Although with marginal independent association, medical students with participation in the MPII study had better mental health than their student colleagues not engaged with this research. Our data pointed out that voluntary participation in a research project which foresees interaction by telephone contact with rheumatic patients, professors, rheumatologists, and colleagues is associated with better mental health.References:[1]Meo SA, Abukhalaf AA, Alomar AA, Sattar K, Klonoff DC. Covid-19 pandemic: Impact of quarantine on medical students’ mental wellbeing and learning behaviors. Pakistan J Med Sci 2020;36(COVID19-S4):S43–8.[2]Gomides A, Ferreira G, Kakehas A, Lacerda M, Marques C, Paiva E et al. Impact of chronic use of antimalarials on SARS-COV-2 infection in patients with immune-mediated rheumatic diseases: protocol design for a multicentric observational cohort in Brazil. JMIR Research Protocols, 2020.PreprintTable 1.Univariate and multivariate analysis of predictors associated to the DASS-21 in undergraduate medical students during the COVID-19 pandemicUnivariate analysisMultivariate analysisVariableB95%CIp-ValueB95%CIp-ValueAge-0.32-0.61 to -0.030.03-0.47-0.81 to -0.130.008Female gender4.883.021 to 6.76---Stable love relationship-2.49-4.35 to -0.640.008-2.5-4.4 to -0.590.01Number of previous comorbidities reported4.693.71 to 5.684.823.73 to 5.92MP-II volunteering-2.81-4.74 to -0.860.005---Worsening in sleep pattern6.414.62 to 8.205.013.07 to 6.96Disclosure of Interests:None declared

Background Atherosclerosis is a chronic inflammatory disease, with interleukin 6 ( IL ‐6) as a major player in inflammation cascade. IL ‐6 blockade may reduce cardiovascular risk, but current treatments to block IL ‐6 also induce dyslipidemia, a finding with an uncertain prognosis. Methods and Results We aimed to determine the endothelial function responses to the IL ‐6–blocking agent tocilizumab, anti–tumor necrosis factor α, and synthetic disease‐modifying antirheumatic drug therapies in patients with rheumatoid arthritis in a 16‐week prospective study. Sixty consecutive patients with rheumatoid arthritis were enrolled. Tocilizumab and anti–tumor necrosis factor α therapy were started in 18 patients each while 24 patients were treated with synthetic disease‐modifying antirheumatic drugs. Forty patients completed the 16‐week follow‐up period. The main outcome was flow‐mediated dilation percentage variation before and after therapy. In the tocilizumab group, flow‐mediated dilation percentage variation increased statistically significantly from a pre‐treatment mean of (3.43% [95% CI, 1.28–5.58] to 5.96% [95% CI, 3.95–7.97]; P =0.03). Corresponding changes were 4.78% (95% CI, 2.13–7.42) to 6.75% (95% CI, 4.10–9.39) ( P =0.09) and 2.87% (95% CI, −2.17 to 7.91) to 4.84% (95% CI, 2.61–7.07) ( P =0.21) in the anti–tumor necrosis factor α and the synthetic disease‐modifying antirheumatic drug groups, respectively (both not statistically significant). Total cholesterol increased significantly in the tocilizumab group from 197.5 (95% CI, 177.59–217.36) to 232.3 (201.62–263.09) ( P =0.003) and in the synthetic disease‐modifying antirheumatic drug group from 185.8 (95% CI, 169.76–201.81) to 202.8 (95% CI, 176.81–228.76) ( P =0.04), but not in the anti–tumor necrosis factor α group. High‐density lipoprotein did not change significantly in any group. Conclusions Endothelial function is improved by tocilizumab in a high‐risk population, even as it increases total cholesterol and low‐density lipoprotein levels.

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.

33-year-old woman presented with inflammatory low back pain for 1 year, recurrent painful oral aphthous, and genital ulcers for 6 months with remaining tissue scarring, extensive pseudofolliculitis on the upper and lower limbs, fever, weight loss, and bilateral anterior uveitis. Seven months later, the pa- tient presented bilateral panuveitis. She had no abdominal pain, diarrhea, anemia, or other symptoms that suggested inflam- matory intestinal disease. Human leukocyte antigen B27 and antinuclear antibody tests were negative. The patient was diag- nosed with Behcet disease according to the International Study Group criteria. 1 Most symptoms were resolved with corticoste- roid eye drops, oral corticosteroids, colchicine, and methotrexate, but the low back pain alleviated only with nonsteroidal anti- inflammatory drugs. After 4 years of follow-up, there is no back pain or skin or eye disease on this treatment. Radiography has not been performed again. Radiography of the sacroiliac joints did not show abnor- malities. After gadolinium administration, coronal T1-weighted magnetic resonance imaging of the sacroiliac joints demonstrated left joint space enhancement indicative of synovitis (Figure, arrows). Edema of the sacral surface of the adjacent sacroiliac joint (Figure, open arrow) and sclerosis of the iliac surface (Figure, asterisk) were also observed, indicating sacroiliitis. Behcet disease, a systemic vasculitis of unknown etiology, is characterized by relapsing episodes of oral and genital ulcers, skin lesions, and ophthalmological disease. It can also affect the vascular, gastrointestinal, and neurological systems. 1Y4 Rheu- matic manifestations have been described in 45% of patients with Behcet syndrome, ranking second after skin and mucosal mani- festations. Inflammatory arthralgia is the most common mani- festation (81% of cases), and sacroiliitis is uncommon (7.5% of cases). 1Y5 Some authors have described a high prevalence of sacroiliitis in patients with Behcet disease, but others have found no association between these conditions. 5 The main reason for these differing results could be a high degree of interobserver variation in interpreting radiographs of the sacroiliac joints. In our case, sacroiliitis was demonstrated by magnetic resonance imaging, the most sensitive method for the detection and early diagnosis of this condition. Joint involvement can be an early manifestation of Behcet syndrome, potentially causing significant

OBJECTIVE: To describe demographic features, disease manifestations and therapy in patients with giant cell arteritis from referral centers in Brazil. METHODS: A retrospective cohort study was performed on 45 giant cell arteritis patients from three university hospitals in Brazil. Diagnoses were based on the American College of Rheumatology classification criteria for giant cell arteritis or temporal artery biopsy findings. RESULTS: Most patients were Caucasian, and females were slightly more predominant. The frequencies of disease manifestations were as follows: temporal headache in 82.2%, neuro-ophthalmologic manifestations in 68.9%, jaw claudication in 48.9%, systemic symptoms in 44.4%, polymyalgia rheumatica in 35.6% and extra-cranial vessel involvement in 17.8% of cases. Aortic aneurysms were observed in 6.6% of patients. A comparison between patients with biopsy-proven giant cell arteritis and those without temporal artery biopsies did not yield significant differences in disease manifestations. All patients were treated with oral prednisone, and intravenous methylprednisolone was administered to nearly half of the patients. Methotrexate was the most commonly used immunosuppressive agent, and low-dose aspirin was prescribed to the majority of patients. Relapses occurred in 28.9% of patients, and aspirin had a protective effect against relapses. Females had higher prevalences of polymyalgia rheumatica, systemic manifestations and jaw claudication, while permanent visual loss was more prevalent in men. CONCLUSIONS: Most of the clinical features of Brazilian giant cell arteritis patients were similar to those found in other studies, except for the high prevalence of neuro-ophthalmic manifestations and permanent blindness in the Brazilian patients. Aspirin had a protective effect on relapses.

Ankylosing spondylitis (AS) is a chronic inflammatory disease. Neurological manifestations can occur because of atlantoaxial subluxation in about 14% of patients1. A 43-year-old man diagnosed with AS according to the 1984 modified New York criteria presented with bulbar symptoms, such as severe dysphagia and dysphonia. Physical examination revealed the left hemitongue paralysis without any other focal neurological finding. He presented stiffness in the cervical, thoracic, and lumbar spine. Magnetic resonance imaging (MRI) …

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.