Vasculitis - Wegener’s granulomatosis in pulmonary practice

Abstract

Introduction and Review of Literature. Diagnosis of Wegener?s granulomatosis in the early stage is difficult because nonspecific symptoms of the disease mimic other disorders. Our Experience. A retrospective review of case records of 37 patients with Wegener?s granulomatosis referred to the Institute for Lung Diseases in Belgrade to be diagnosed and treated over a 15-year period was carried out. There were 37 patients (20 males and 17 females), ranging in age from 18 to 73 years (mean age 46.2 years). The mean period from the onset of first symptoms to diagnosis of Wegener?s granulomatosis was 4.59?6.15 months. The American College of Rheumatology criteria were fulfilled in all patients. Twenty-five of 37 patients had evidence of systemic, generalized form of Wegener?s granulomatosis and a limited involvement of the upper and lower respiratory system occurred in 12 cases. The frequency of different system involvement expressed in percents was as follows: the upper respiratory tract 64.8%, the lower respiratory tract 100%, the kidneys 67.5%, the musculoskeletal system 40.5%, the skin 27.2%, the eyes 8.1%, the nervous system in two patients. Anti-neutrophil cytoplasmic antibodies test was positive in 32 (6.5%) patients, and negative in 5 (13.5%). All patients were antinuclear antibodies negative. Histological evidence of granulomatous vasculitis was obtained in 34 (91.9%), whereas in three patients the diagnosis was based on clinical manifestations and positive c-anti-neutrophil cytoplasmic antibodies test.