In 50 of 94 patients with bronchiolitis obliterans we found no apparent cause or associated disease, and the bronchiolitis obliterans occurred with patchy organizing pneumonia. Histologic characteristics included polypoid masses of granulation tissue in lumens of small airways, alveolar ducts, and some alveoli. The fibrosis was uniform in age, suggesting that all repair had begun at the same time. The distribution was patchy, with preservation of background architecture. Clinically, there was cough or flu-like illness for 4 to 10 weeks, and crackles were heard in the lungs of 68 per cent of the patients. Radiographs showed an unusual pattern of patchy densities with a "ground glass" appearance in 81 per cent. Physiologically, there was restriction in 72 per cent of the patients, and 86 per cent had impaired diffusing capacity. Obstruction was limited to smokers. The mean follow-up period was four years. With corticosteroids, there was complete clinical and physiologic recovery in 65 per cent of the subjects; two died from progressive disease. This disorder differs from bronchiolitis obliterans with irreversible obstruction. It was confused most often with idiopathic pulmonary fibrosis. In view of the benign course and therapeutic response, a histologic distinction is important.
Patients with confirmed interstitial pneumonia were initially classified histologically into "desquamative" (n = 40) and "usual" (n = 53) types, and followed for one to 22 years. Both the diagnosis and the extent of fibrosis affected the course and response to therapy. Mortality in desquamative interstitial pneumonia was 27.5 per cent, and mean survival 12.2 years, as compared with 66.0 per cent and 5.6 years in usual interstitial pneumonia (P less than 0.01). Without treatment, 21.9 per cent with the desquamative but none with the usual type improved. With corticosteroid therapy, 61.5 per cent with desquamative and only 11.5 per cent with usual interstitial pneumonia improved, whereas 27.0 per cent and 69.2 per cent worsened. We conclude that the histologic classification of chronic interstitial pneumonia used here permits forecasts of prognosis and response to treatment that cannot be deduced from other data.
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