A patient with idiopathic pulmonary fibrosis may develop an accelerated form of the disease. A patient with mild or moderate pulmonary fibrosis may deteriorate and die during such an episode. The precipitating cause for the episode is usually unknown.
Features:
(1) acute progression of dyspnea over 1 to 4 weeks
(2) development of new, diffuse opacities in the chest X-ray
(3) worsening hypoxemia
(4) rapid development of respiratory failure
(5) exclusion of acute infection or other causes of the deterioration
(6) lung biopsy shows acute alveolar injury with or without formation of hyaline membranes
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