抄録
Forty-One year old man who was admitted to the Tokyo University Branch Hospital on suspicion of bronchogenie carcinoma, was diagnosed as histiocytosis-X at the beginning of the course which followed with a remission of two and a half years after administration of FAMT, and died acutely in spite of the readministration of the drugs 4 months after the occurence of relapsus. Based on the histopathological examination upon autopsy, it was diagnosed as Wegener's granulomatosis (pulmonary type).
Several unusual clinical manifestation were noted at the begining, that is, subileus condition, diabetic state, hepatomegaly with liver function disturbance, but cough and sputum were minimum through the course. At necropsy, small scarred xanthogranulomas—coincident with chest X-ray shadow which had responded well to FAMT therapy—accompanied with thromboangitis locating central part, and the kidney showed typical finding of local glomerulonephritis. The main cause of death, however, were thought to be necrotizing phlegmonous colitis and diffuse liver necrosis both of which followed to phlebothrombosis of portal system.
The characteristic cytological findings of sputa, pleural fluid and transcutaneous needle biopsy of the lung were described, and chest X-ray finding of Wegener's granulomatosis and its treatment with immunosuppresive agents were discussed.