Abstract Background: Little has been published about the epidemiology of Granulomatosis with polyangiitis (GPA) in South America, especially in the intertropical zone, and no epidemiological data from Brazil are available. The purpose of the present study was to draw a clinical and demographic profile of GPA patients living in Northeastern Brazil based on laboratory, histological and imaging findings, and evaluate the frequency of organic involvement. Methods: Clinical, epidemiological and treatment data of GPA patients were collected retrospectively and compared with the literature. Results: The cohort included 25 GPA patients (84% female) aged 45.8 ± 16.1 years. Renal and ear-nose-throat (ENT) manifestations were the most common (both 64%). One third (32%) of the patients had 24-h proteinuria > 1 g, 50% had creatinine clearance < 50 mL/min at the time of diagnosis, and 33% had recurrent kidney damage during disease progress. The affected organs included lungs (60%), joints (44%), skin (32%), peripheral nervous system (28%), eyes (28%) and heart (16%). ENT involvement (n = 16/64%) was less frequent in our region than in São Paulo (n = 115/85.8%). Renal (n = 16/64%) and pulmonary (n = 15/60%) involvement was less frequent in our region than in the U.K. (renal n = 30/90%; pulmonary n = 28/84.8%). Conclusion: Most of our patients were female, presented the generalized form and were diagnosed late. The frequency of the main clinical manifestations (ENT, renal and pulmonary) was lower than that observed at higher latitudes, suggesting the existence of a Northeast Brazilian clinical and epidemiological profile and adding to our knowledge of this rare condition.

FUNDAMENTO: Embora haja diversos estudos epidemiológicos publicados referentes à artrite de Takayasu (AT), nenhum analisou a influência do gênero nas manifestações clínicas e laboratoriais ou as alterações vasculares no início da doença. OBJETIVO: Analisar a influência do gênero nas manifestações clínicas e laboratoriais e nas variações de imagiologia vascular no início da AT. MÉTODOS: Estudo de coorte retrospectivo, unicêntrico que avaliou 55 pacientes consecutivos com AT entre 1982 e 2012. Todos os dados clínicos e resultados de testes laboratoriais relacionados ao início da doença foram analisados. Incluímos apenas pacientes de 12 a 35 anos no diagnóstico para excluir fatores relacionados à idade. RESULTADOS: Analisamos 17 homens e 38 mulheres, sendo a maioria caucasiana, com uma média de idade comparável entre os gêneros. Não houve diferença de gênero referente às características clínicas ou laboratoriais, comorbidades ou tabagismo, exceto pela dor abdominal, que apresentou ser mais comum em homens. Com relação às lesões vasculares, a presença de aneurismas da aorta ascendente foi significativamente mais frequente em homens. O gênero masculino representou um fator de risco independente para a ocorrência de dor abdominal e aneurismas da aorta ascendente em pacientes com AT. CONCLUSÃO: A dor abdominal e aneurismas da aorta ascendente ocorreram com mais frequência em homens com AT, sugerindo um perfil de doença mais severa em homens.

BACKGROUND: Although there are various published epidemiological studies regarding Takayasu's arteritis (TA), none have analyzed the influence of gender on the clinical and laboratory manifestations or vascular alterations at disease onset. OBJECTIVES: To analyze the influence of gender on clinical and laboratory manifestations and variations in vascular imaging at TA onset. METHODS: A retrospective, unicentric cohort study that evaluated 55 consecutive TA patients between 1982 and 2012. All available clinical data and laboratory test results related to the onset of the disease were analyzed. We included only patients aged 12-35 years at diagnosis to exclude age-related factors. RESULTS: We analyzed 17 men and 38 women, mostly Caucasian, with a comparable mean age between genders. There was no gender difference regarding the clinical or laboratory characteristics, comorbidities, or smoking habit, except for abdominal pain, which was more common in men. Regarding vascular lesions, the presence of ascending aortic aneurysms was significantly more frequent in males. Male gender represented an independent risk factor for the occurrence of abdominal pain and ascending aortic aneurysms in TA patients. CONCLUSION: Abdominal pain and ascending aortic aneurysms occurred more frequently in men with TA, suggesting a more severe disease profile in males.