Wegener's granulomatosis: prevalence of the initial clinical manifestations--report of six cases and review of the literature.
OBJECTIVE
To describe the initial clinical manifestations of Wegener's Granulomatosis (WG) in Brazil.


METHODS
Retrospective analysis of six medical records of WG patients followed-up at the Rheumatology Department of Hospital Geral of Fortaleza (HGF), as well as a bibliographic survey of cases of WG in Brazil on LILACS, SciELO, and MEDLINE databases.


RESULTS
The study identified 49 patients, 15 (31%) males and 34 (69%) females. Systemic disease was observed in 35 patients (73%): 28 adults, 5 children, and 2 teenagers. Limited disease was observed in 13 adults and 1 child. The average age of onset in adults was 42.2 years (18 to 65 years). Acute clinical manifestations, with the onset of symptoms less than three months before the diagnosis, were observed in 41% (20/49) of the patients, and the insidious presentation in 59% (29/49) of the patients. The prevalence of the initial clinical manifestations in adults with systemic disease (n = 28) was 64% (18/28), upper airways, 36% (10/28), lungs, 18% (5/28), kidneys, 25% (7/28), eyes, 11% (3/28) skin, 25% (7/28), musculoskeletal, and 7% (2/28), neurological. In adults (n = 13) with limited disease, prevalent symptoms included: upper airway, 84% (10/13), eyes, 23% (3/13), and lungs, 15% (2/13).


CONCLUSIONS
The prevalence of the initial clinical manifestations of WG in Brazil was similar to that reported in the literature. The lack of specific symptoms may delay diagnosis cases with insidious presentation of the disease and increase the morbidity and mortality in acute disease.
Serviço de Reumatologia, Hospital Geral de Fortaleza. carlosewerton@hotmail.com
Carlos Ewerton Maia
CE
Rodrigues
Maria Roseli Monteiro
MR
Callado
Christiane Aguiar
CA
Nobre
Francisca Edwiges Araújo
FE
Moura
Rejane Maria Rodrigues de Abreu
RM
Vieira
Lucas Alverne Freitas
LA
de Albuquerque
Walber Pinto
WP
Vieira
D000293:Adolescent; D000328:Adult; D003430:Cross-Sectional Studies; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000150:complications / Q000175:diagnosis*; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D055815:Young Adult