The authors report the clinical, radiological and histological finding
s in a 63-year-old male patient who developed severe necrotizing bronc
hitis, necrotizing angiitis, and secondary amyloidosis of the right up
per love and intermediate bronchus. The patient expired due to respira
tory insufficiency. At the age of 27 years, the patient had had radiot
herapy of the mediastinum because of suspected Hodgkin's disease. Acut
e pneumonia suggestive of Q-fever infection was diagnosed at the age o
f 48. Progressive restrictive lung disease developed during the last d
ecade. 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 effusion
s displayed numerous activated T lymphocytes. Analysis of endogenous l
ectins revealed alterations of the pulmonary defense system. The clini
cal history, histological and immunological findings suggest that chro
nic Q fever may induce remarkable changes in the immune system, compar
able to autoimmune-reactive diseases.