Wegener's granulomatosis

Symptoms, diagnosis and treatment of five cases

Abstract

Introduction: Wegener’s granulomatosis (WG) is a type of systemic granulomatous vasculitis primarily affecting the respiratory tract and kidneys.
Objectives: to study the signs and symptoms presented by patients cared for at Clinica Médica "C" of the Clinicas Hospital.
Methods: we conducted a descriptive, restrospective study including patients with a clinical and anatomopathological definitive diagnosis of WG. Five cases, diagnosed between 2002 and 2006, were analysed.
Results: average age of diagnosis was 51 years old (SD ± 24,5). Affection of the upper and lower respiratory tract was the most frequent clinical finding. Initial diagnosis in the 5 cases was respiratory tract infection. They all evidenced anticytoplasm of the neutrophil antibodies. (ACNA). Diagnosis was confirmed by a biopsy of the respiratory tract. Two patients developed malignant neoplasia prior to the diagnosis of WG. Average follow-up was 20 months after the diagnosis. Induction treatment of methylprednisolone and cyclophosphamide, and follow-up treatment with prednisone and azathioprine, were applied.
Conclusions: WG may be interpreted as a respiratory tract infection in its initial stage, when thorax tomography and positive ACNA findings constitute essential factors for diagnosis, and thus enable the orientation of biopsies toward "symptomatic sites" and the early initiation of immunosuppressor treatment.