ACUTE EXACERBATION IN IDIOPATHIC PULMONARY FIBROSIS - ANALYSIS OF CLINICAL AND PATHOLOGICAL FINDINGS IN 3 CASES

We treated three patients with idiopathic pulmonary fibrosis who had a n acute clinical exacerbation. We analyzed their clinical, radiographi c, therapeutic, and pathologic findings. Their initial symptoms were i nfluenza-like illness or cough with fever, and all had leukocytosis an d elevation of C-reactive protein. Infectious events were ruled out by extensive bacteriologic and serologic examination. The patients' lung injury scores progressed rapidly to severe lung injury compatible wit h adult respiratory distress syndrome. Findings from bronchoalveolar l avage fluid showed marked neutrophilia and elevation of albumin concen trations. All patients showed various degrees of improvement following corticosteroid therapy. Histologic findings from open lung biopsy spe cimens showed both usual interstitial pneumonia (UIP) and organizing a cute lung injury pattern. Whether these two forms of interstitial pneu monia (UIP and acute lung injury pattern) are variants of one disease or are unrelated and also the effectiveness of corticosteroid therapy on such conditions remain to be determined by further studies.