Rapidly progressing respiratory insufficiency of uncertain etiology

: In a 59-year-old male patient, chronic dry cough and dyspnoea on exertion preexisting for several years became rapidly progressive within a few weeks prior to hospitalisation. He died one month after admission from respiratory failure. Three months before admission, history, pulmonary function tests, and computed tomography (CT) of the chest revealed no evidence of asthma, COPD, or any other lung disease. Clinical examination showed no clubbing, but end-inspiratory velcro-rales were audible over both lungs. Inhaled steroids and diuretics did not bring clinical amelioration. On admission there were basal consolidations, bronchiectases, and predominant fibrotic changes with honeycombing and subpleural thickening over both lungs, in the absence of any ground-glass pattern in the CT. At the same time lymphocytosis predominated in bronchoalveolar lavage (BAL). The search for pneumonia, viral infection, tumour, vasculitis, or a drug-related disorder remained negative. Pathological examination at autopsy showed nonuniform fibrosing alveolitis.