title: 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.
authors.affiliation: Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo, Av. Dr. Arnaldo, 455, 3 degrees andar, Sala 3190, CEP 01246-903 São Paulo, Brazil.
authors.forename: Fernando Henrique Carlos
authors.initials: FH
authors.lastname: de Souza
authors.forename: Ari Stiel
authors.initials: AS
authors.lastname: Radu Halpern
authors.forename: Carmen Silvia
authors.initials: CS
authors.lastname: Valente Barbas
authors.forename: Samuel Katsuyuki
authors.initials: SK
authors.lastname: Shinjo
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000328:Adult; D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D001938:Brazil; D002648:Child; D003520:Cyclophosphamide / Q000627:therapeutic use*; D005260:Female; D005500:Follow-Up Studies; D014890:Granulomatosis with Polyangiitis / Q000188:drug therapy* / Q000276:immunology / Q000503:physiopathology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007596:Joints / Q000503:physiopathology; D007668:Kidney / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D009420:Nervous System / Q000503:physiopathology; D012137:Respiratory System / Q000503:physiopathology; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000305:Adrenal Cortex Hormones; D019268:Antibodies, Antineutrophil Cytoplasmic; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide