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.
A retrospective study of 152 cases of lymphomatoid granulomatosis (LYG) was undertaken to expand current knowledge about clinical manifestations and therapy, and to identify prognostic indicators in this disease. The major clinical and radiographic features and extra-pulmonary sites of involvement noted in previous studies were essentially confirmed in our study. Adverse prognostic factors included neurologic manifestations, and large numbers of atypical lymphoreticular cells within the pulmonary infiltrate. Unilateral chest lesions and large numbers of small lymphocytes and histiocytes within the infiltrate were associated with a better prognosis. Malignant lymphoma involving lymph nodes developed in 12% of patients. Almost two-thirds of patients have died and the median survival was only 14 months. No particular mode of therapy was found to be satisfactory for LYG, although there was some suggestion that corticosteroids may be useful. Randomized prospective studies are necessary for definitive recommendations regarding therapy. Cancer 43:360-373, 1979. YMPHOMATOID GRANULOMATOSIS (LYG) is L a unique form of pulmonary angiitis and granulomatosis which was first described in 1972 by Liebow et al. 17319 Histologically it is a necrotizing angiocentric and angiodestructive infiltrative process composed of small lympho-cytes, plasma cells, histiocytes and atypical lympho-reticular cells. It usually presents in middle age with bilateral pulmonary infiltrates ; skin, nervous system, and kidney are commonly involved, and the mortality is high. Since the initial report which included 40 patients , only a few additional cases of LYG have been reported in the literature'~2~3~7~8~"2.'5.'6.-22,253-33.35 and many questions remain un-answered regarding therapy, prognosis and natural history. The purpose of this paper is to expand the current knowledge about clinical manifestations and response to therapy in a large number of patients, and to identify clinical and pathologic features which may be useful prognostic indicators in this disease. MATERIALS A N D METHODS One hundred and fifty-seven patients with LYG were identified in the consultation files of A. A. Liebow. All of the cases had been referred to Dr. Liebow for consultation; 36 were included in the first description of LYG,lS and 12 others were subsequently pub-hematoxylin and eosin stained microscopic slides of biopsy or autopsy material were available for review in each case; paraffin blocks and wet tissue were available in some and a variety of special stains were done in many. Five of the patients were excluded from the present study because they manifested only extrapulmonary involvement (skin, central nervous system, tongue, nasal cavity and naso-pharynx, liver and mesentery), without evidence of lung involvement. Clinical data and follow-up information were obtained from the referring physicians or the patients themselves. Since we did not have access to hospital charts, details of clinical and laboratory data were not always complete. lished e l s e w h e r e. ...
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.