Wegener’s granulomatosis: experience from a Brazilian tertiary center

Abstract

Most epidemiological studies with Wegener's granulomatosis (WG) patients are based on populations from the Northern hemisphere, whereas very few studies have been conducted in Southern hemisphere populations, particularly from South America. The authors performed a large retrospective, demographic study including clinical and laboratory profiles of 134 consecutive WG patients seen at one Brazilian center from 1999 to 2009. Mean age at initial WG diagnosis was 43.4 +/- 15.5 years, and mean disease duration was 8.6 +/- 6.6 years. Sixty-four (47.8%) patients were male and a total of 113 (84.3%) subjects were white. Ear/nose/throat involvement occurred in 85.8%. The classic lung and renal involvement were observed in 77.6% and 75.4%, respectively, followed by ocular (35.8%), musculoskeletal (33.4%), cutaneous (29.1%), neurological (20.1%), cardiac (11.2%), and genitourinary involvement in 2.2% of cases. Cytoplasmic pattern-antineutrophil cytoplasmic antibody was detected in 83 (61.9%) cases. Ten (7.5%) individuals presented limited forms of WG. Classic therapy with corticosteroids and cyclophosphamide was used in 97 cases (72.4%). There were no cases of tuberculosis or Pneumocystis jiroveci pneumonia, but cutaneous herpes zoster occurred in eight (6.0%) individuals. There were 29 deaths (21.6%). Eighteen patients died of septic shock (mainly bacterial pneumonia), whereas four died of alveolar hemorrhage, four of myocardial infarction, and three of other causes. In summary, our data from a very large retrospective and descriptive study mirrored the main clinical features of WG described in other countries, demonstrating that they may serve as a reference for South American populations.

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

“…Compared to the international literature, the upper respiratory tract and the lungs were less frequently affected in our patients from Northeastern Brazil [11,13,25]. The most frequent disease extension was generalized, and time to diagnosis was relatively long (in one case, 18 years from the onset of symptoms).…”

“…The only two Brazilian studies investigating the clinical and laboratory profile of GPA patients reported a spectrum of clinical manifestations similar to that found in the international literature [13,14]. However, these Brazilian studies on GPA were conducted outside the intertropical zone [13,14].…”

“…For example, the female gender was predominant in a study from Southern Europe while the male gender was predominant in a study from Northern Europe [5,10]. Within the southern hemisphere, the male gender is predominant in New Zealand (43°S) (M/F ratio: 1.2) and Argentina (31°S) (M/F ratio: 1.2), while the female gender is predominant in Chile (33°S) (M/F ratio: 0.8) and São Paulo (23°S) (M/F ratio: 0.9) [11,13,15,16]. In short, available data suggest a tendency for female predominance among GPA patients at low latitudes in both hemispheres [10].…”

“…-ENT involvement was less frequent in Northeastern Brazil (n = 16; 64%) than in São Paulo (n = 115; 85.8%) and New Zealand (n = 63; 86%) [11,13]. -Pulmonary involvement was less frequent in Northeastern Brazil (n = 15; 60%) than in the U.K.…”

“…Little has been published about the epidemiology of GPA in South America, especially in the intertropical zone, and no epidemiological data from Brazil are available [13][14][15][16][17]. The only two Brazilian studies investigating the clinical and laboratory profile of GPA patients reported a spectrum of clinical manifestations similar to that found in the international literature [13,14].…”