Fibrosing alveolitis. Causes, characteristics, and consequences.

Fibrosing alveolitis may be cryptogenic (idiopathic) or secondary to a wide variety of disease processes. In almost all cases, acute and subacute inflammatory lesions progress to fibrosis and finally to a terminal state called honeycomb lung. Diagnosis is based on clinical and radiologic findings together with demonstration of typical pathologic changes on lung biopsy. The biopsy specimen should be large and taken from less severely involved lung areas to increase the probability that early, more specific lesions will be harvested. Prednisone is usually recommended for specific therapy. Therapy is a factor in duration of survival, although patient response varies.