Allergic granulomatosis and angiitis (Churg-Strauss syndrome). Report of an autopsy case in a nonasthmatic patient.

A 54-year-old nonasthmatic man was found to have allergic granulomatosis and angiitis (AGA) (Churg-Strauss syndrome) with pulmonary lesions suggestive of diffuse panbronchiolitis (DPB) at autopsy. The patient, with initial symptoms of cough and sputum, developed progressive dyspnea, eosinophilia, emaciation, fever, mononeuritis multiplex and myocardial infarction. The hypereosinophilic syndrome (HES) and DPB were suspected clinically. Corticosteroid therapy was not given at any time during the course. Autopsy revealed necrotizing, granulomatous angiitis affecting medium-sized arteries in many organs, extravascular granulomas in the interstitium of the heart and tissue infiltration by eosinophils. The heart showed widespread myocardial fibrosis and small foci of muscle fiber coagulation necrosis, which seemed to be the cause of death. In the lungs, the walls of respiratory bronchioles showed marked thickening with lymphocytic infiltration, lymph follicle formation and fibrosis. Accumulation of xanthoma cells was also observed. On the basis of the findings of clinical and pathological examinations, the patient was considered to have had DPB before the development of AGA.