A study of Wegener's granulomatosis

Abstract

164 cases with Wegener's granulomatosis (WG) in Japan for 20years since 1958 up to 1977 based on the Annual of the pathological Autopsy Cases in Japan and six patients with WG which we have experienced in our institute were observed. The diagnosis of six patients with WG was made on clinical symptoms and the histological examination of the biopsied materials from the nasal membrane, bronchus and kidney.In our clinic, the male-tofemale ratio was 1 to 1 and the mean age at the onset of the initial manifestations of WG was 43.7years (range: 15.8 to 72.5years). Fever and upper respiratory symptoms were the first manifestations of WG in the majority of our cases. In the clinical features during the course of WG, upper respiratory and urinary manifes-tations were the most common (six cases). Pulmonary involvement was noted in five cases, eye in three cases, ear in one case, and central nervous system in one case. When the patients were given no drugs abnormal hematological manifestations were found: increase of erythrocyte sedimentation rate, C-reactive protein positive (5+_??_6+), neutrophilia and α2-globulinemia in all cases, γ-globulinemia and increase of ALP in five cases, leukocytosis in four cases, hypoalbuminemia in three cases. Positive rheumatoid factor were seen in 50%, increase of IgG and IgA in four patients, and hype rcomplementemia in four ones. The abnormalities in T cell function, such as the decrease of T cell counts, impaired skin delayed hypersensitivity (PPD skin test), and reduced lymphocyte blastogenesis by PHA, were noted in died cases.In autopsy cases of WG, the male-to-female ration was 84 to 80 and their ages ranged from 10 to 68years (the mean of 39.0years), The incidences of the organ system involvement in WG were given in Table 5. Upper respiratory tracts involvement was noted in 69.5% of 164 autopsy cases, pulmonary involvement in 47.0%, kidney in 43.9%, spleen in 23.8%, liver in 14.0% and so on.These observations were discussed with reference to the reports in the literature.