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.

BackgroundFrosted branch angiitis is a rare, severe condition. It can be either a primary or a secondary condition and is characterized by rapid deterioration of vision and fulminant retinal vasculitis that manifests as diffuse sheathing of retinal vessels, macular edema, papillitis, vitritis and anterior uveitis. We aimed to describe a case of frosted branch angiitis and cerebral venous sinus thrombosis as an initial neuro-Behçet’s disease onset. Diagnosis of Behçet’s disease was based on the current 2014 International Criteria for Behçet’s Disease and the International consensus recommendation criteria for neuro-Behçet’s disease. In addition, a literature review using search parameters of “frosted branch angiitis”, “Behçet” and “neuro-Behçet” in the PubMed database is presented.Case presentationA 28-year-old Brazilian pardo woman presented to our hospital with abrupt bilateral vision loss associated with recurrent aphthous oral ulcers 6 months before visual symptom onset. A fundus examination showed bilateral widespread retinal vasculitis with venous and arterial white sheathing, optic disc swelling, macular edema, and retinal hemorrhages, leading to the diagnosis of frosted branch angiitis. An extensive systemic workup for retinal vasculitis was uneventful, except for brain magnetic resonance imaging demonstrating cerebral venous sinus thrombosis and lymphocytic aseptic meningitis. A diagnosis of neuro-Behçet’s disease was made, and treatment was started with methylprednisolone therapy 1 g/day for 5 consecutive days, followed by oral mycophenolate mofetil and infliximab 5 mg/kg infusion. The patient’s response was rapid, with improvement of visual acuity to hand movement and counting fingers by day 7 and final visual acuity of counting fingers and 20/130.ConclusionsFrosted branch angiitis may be associated with infectious, noninfectious, or idiopathic causes. An extensive workup should be done to exclude systemic vasculitis such as Behçet’s disease. Treatment with systemic steroids must be promptly initiated in association with specific treatment aimed at inflammation control and blindness risk reduction.

The recommendations proposed by the Sjögren's Syndrome Committee of the Brazilian Society of Rheumatology for the treatment of Sjögren's syndrome were based on a systematic review of literature in Medline (PubMed) and the Cochrane databases until October 2014 and on expert opinion in the absence of studies on the subject. 131 items classified according to Oxford & Grade were included. These recommendations were developed in order to guide the appropriate management and facilitate the access to treatment for those patients with an appropriate indication, considering the Brazilian socioeconomic context and pharmacological agents available in this country.

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Purpose of the present paper is to point out the design, development and deployment of the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to pediatric and adult patients with Behçet’s disease (BD). The Registry is a clinical physician-driven non-population- and electronic-based instrument implemented for the retrospective and prospective collection of real-life data about demographics, clinical, therapeutic, laboratory, instrumental and socioeconomic information from BD patients; the Registry is based on the Research Electronic Data Capture (REDCap) tool, which is thought to collect standardised information for clinical real-life research, and has been realised to change over time according to future scientific acquisitions and potentially communicate with other existing and future Registries dedicated to BD. Starting from January 31st, 2021, to February 7th, 2022, 110 centres from 23 countries in 4 continents have been involved. Fifty-four of these have already obtained the approval from their local Ethics Committees. Currently, the platform counts 290 users (111 Principal Investigators, 175 Site Investigators, 2 Lead Investigators, and 2 data managers). The Registry collects baseline and follow-up data using 5993 fields organised into 16 instruments, including patient’s demographics, history, clinical manifestations and symptoms, trigger/risk factors, therapies and healthcare access. The development of the AIDA International Registry for BD patients will facilitate the collection of standardised data leading to real-world evidence, enabling international multicentre collaborative research through data sharing, international consultation, dissemination of knowledge, inclusion of patients and families, and ultimately optimisation of scientific efforts and implementation of standardised care.Trial registration NCT05200715 in 21/01/2022.

ObjectivesRecommendations of Brazilian Society of Rheumatology for the treatment of Sjögren's syndrome (SS) were developed to guide management of SS considering the Brazilian social and economic context.MethodsIt was based on specialist's opinion and systematic review on MEDLINE (PubMed) and Cochrane database until October 2014, including 127 articles classified according Oxford & Grade.ResultsForty-four recommendations were organized in 3 main topics: Part 1. General recommendations and patient education: The management of SS should be conducted by multidisciplinary team. Systemic treatment should be according disease severity measured by EULAR Sjögren's Syndrome Disease Activity (ESSDAI). Patients should avoid caffeine, tobacco, alcohol, toothpaste with abrasive, and mouthrinses with alcohol. Patient should be educated about preventive measures for oral health and hydration. Aerobic exercise improves fatigue and quality of life. Immunization to influenza and pneumococo are indicated. Serum levels of vitamin D should be evaluated and supplemented if it is necessary. Part 2. Symptomatic treatment of dryness: Topical treatment for dry mouth includes saliva substitutes, sugar-free candies and gums. Topical treatments for dry eye are lubricants (glucanes or carboximethylcelulose), cyclosporine 0.05%, and punctual occlusion. Topical glucocorticoids may be used for severe dry eye for short time avoiding complications. Pilocarpine and cevimeline should be used for dry mouth and for severe dry eye. N-acetylcysteine may be used for dryness symptoms, including patients showing intolerance for muscarinic agonists. Omega-3 supplementation may be used to dry eye. Part 3. Systemic treatment: Immunosupressant and/or biological therapy are not indicated to dryness treatment. Hydroxichloroquine, glucocorticoid, and immunossupressants (azathioprine, mycophenolate mofetil, cyclophosphamide, cyclosporine) should be indicated according severity of systemic involvement. Rituximab is indicated to treat systemic manifestations without improvement with immunosuppressive therapy. Abatacept and belimumab may be considered in patients not responding to rituximab and with high level of disease activity.AcknowledgementsSociedade Brasileira de Reumatologia (SBR)Disclosure of InterestNone declared

As infecções virais são causas conhecidas de manifestações reumatológicas, geralmente como diagnóstico diferencial de poliartrites agudas. Neste artigo, abordaremos os aspectos clínicos, diagnósticos e de tratamento de algumas viroses comuns na prática clínica, que devem ser reconhecidas pelo reumatologista, tanto pelas manifestações articulares quanto autoimunes. Unitermos: Arbovírus. Parvovírus B19. Herpes-vírus. Artrite. Autoimunidade.

A sarcoidose é uma doença inflamatória multissistêmica, com apresentação e curso clínico variável, podendo afetar qualquer órgão e evoluir desde remissão espontânea até disfunção de múltiplos órgãos. Essa diversidade clínica, associada à semelhança com outras doenças mais comuns, muitas vezes leva à incerteza diagnóstica e atrasos no tratamento. Com isso, o diagnóstico da sarcoidose é desafiador, mas pode ser baseado em três critérios: apresentação clínica compatível; evidência de granulomas não caseosos; e exclusão de diagnósticos alternativos. No entanto, mesmo quando todos os critérios são preenchidos, a probabilidade de diagnóstico de sarcoidose varia de definitiva a apenas possível, dependendo da presença de sinais radiológicos e histopatológicos mais ou menos característicos. O diagnóstico de sarcoidose pode ser difícil na ausência de manifestações torácicas ou cutâneas e a busca pela evidência histopatológica nem sempre é fácil. Além disso, diagnósticos diferenciais, principalmente tuberculose, devem ser considerados. Neste artigo, discutimos os principais pontos a serem considerados nesse trabalho diagnóstico e propomos um algoritmo para sistematizar o diagnóstico dessa patologia. Unitermos: sarcoidose; diagnóstico; granuloma; biomarcadores; diagnóstico diferencial.