The authors report the clinical, radiological and histological findings in a 63-year-old male patient who developed severe necrotizing bronchitis, necrotizing angiitis, and secondary amyloidosis of the right upper love and intermediate bronchus. The patient expired due to respiratory insufficiency. At the age of 27 years, the patient had had radiotherapy of the mediastinum because of suspected Hodgkin's disease. Acute pneumonia suggestive of Q-fever infection was diagnosed at the age of 48. Progressive restrictive lung disease developed during the last decade. Serological evaluation revealed IgM and IgA high titers against Coxiella burnetii. IgA, complement and amyloid deposits were detected in the walls of small arteries. Bronchial lavage and pleural effusions displayed numerous activated T lymphocytes. Analysis of endogenous lectins revealed alterations of the pulmonary defense system. The clinical history, histological and immunological findings suggest that chronic Q fever may induce remarkable changes in the immune system, comparable to autoimmune-reactive diseases.