Granulomatosis with polyangiitis in Northeastern Brazil: study of 25 cases and review of the literature

Abstract

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.