Abstract
Twenty-three patients, including three children, with pulmonary alveolar proteinosis (PAP) were studied over a 15 year period. Based on the overall course and outcome they were divided into two groups. Group I (24%) had spontaneous remission and received no treatment. Group II (76%), because of progressive dyspnea and deterioration of pulmonary function tests, underwent lung lavage. Of those who underwent lung lavage and were followed, 76% had a favorable response (Group II-a) and a significant number of them developed a complete remission, while 21% (Group II-b) had no response to lung lavage. Group I had higher arterial PO2 and lower alveolar-arterial oxygen partial pressure difference [P (A-a)O2] than Group II (72±5 and 38±3 vs 57±4 and 51±3,p<0.05). Similarly, the values of arterial PO2 and P(A-a)O2 were better in Group II-a than Group II-b (57±4 and 51±3 vs 40±6 and 65±11). In lavage material of 16 patients studied, macrophages appeared unusually enlarged and their number (% of differential count) was significantly lower (60±4%) than normal (90–95%). In contrast to previous reports, there was no evidence of active infection related to unusual or opportunistic organisms. No death related to PAP was observed. This prospective long term follow-up of large series of patients with PAP documents the overall course, prognosis and management of this illness.
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Kariman, K., Kylstra, J.A. & Spock, A. Pulmonary alveolar proteinosis: prospective clinical experience in 23 patients for 15 years. Lung 162, 223–231 (1984). https://doi.org/10.1007/BF02715650
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DOI: https://doi.org/10.1007/BF02715650