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

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%).

Demographic and Clinical Features of ANCA-Associated Vasculitides in a Peruvian Tertiary Center

“…Likewise, as to MPA and EGPA, our findings are similar to those from other Latin American, European, and Asian studies; that is, MPA patients were the oldest, 4,5,8,13,16,23,25,26,28,29 whereas EGPA patients tended to be the youngest 8,13,16,23,29 . However, as to GPA, Colombian patients and our patients were older (59 [14.8] and 53.4 [14.1] years, respectively) than those reported from Mexico and Brazil (41.9 to 45.8 years) 6,8,13,14,24 . This might be because of the difficulties these patients may have in seeking health care or be referred to rheumatology because the presenting symptoms are not specific (eg, sinusitis) and can be interpreted as caused by some other condition.…”

“…2,[18][19][20] We have observed the opposite with a female predominance; that has also been the case in studies from other Latin American countries such as Chile, Brazil, Argentina, Colombia, and Mexico. [4][5][6]8,13,14,21 It is also worth to note that, in the majority of these studies as well as in ours and in those from Japan and China, 15,16,22 the female predominance was particularly observed in MPA. On the other hand, male sex predominated in our EGPA patients as well as in studies from Mexico, the United States, and China 8,16,23 ; however, in Brazil, the female sex was slightly more frequent in this group.…”

“…ANCA-associated vasculitides have been described as occurring more frequently in males; these data, however, comes primarily from European studies 2,18–20 . We have observed the opposite with a female predominance; that has also been the case in studies from other Latin American countries such as Chile, Brazil, Argentina, Colombia, and Mexico 4–6,8,13,14,21 . It is also worth to note that, in the majority of these studies as well as in ours and in those from Japan and China, 15,16,22 the female predominance was particularly observed in MPA.…”

“…Currently, there are few studies about the features of AAV in Latin American populations; these studies include, by and large, a relatively small number of patients 4–8 . Moreover, there are no studies of this nature in Peruvians.…”

“…[3][4][5] Currently, there are few studies about the features of AAV in Latin American populations; these studies include, by and large, a relatively small number of patients. [4][5][6][7][8] Moreover, there are no studies of this nature in Peruvians. Therefore, we aimed at identifying the demographic and clinical features of patients with AAV using the database of a Peruvian tertiary referral hospital.…”