Chapter

Pulmonary alveolar proteinosis

D.J. Hendrick and S.J. Bourke

in Oxford Textbook of Medicine

Fifth edition

Published on behalf of Oxford University Press

ISBN: 9780199204854
Published online May 2012 | e-ISBN: 9780199570973 | DOI: http://dx.doi.org/10.1093/med/9780199204854.003.181407_update_001

Series: Oxford Textbooks

Pulmonary alveolar proteinosis

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Pulmonary alveolar proteinosis is caused by failure, usually due to autoimmune antibodies, of GM-CSF to stimulate surfactant catabolism by alveolar macrophages. Presentation is with progressive shortness of breath, or with pneumonic illness due to superimposed infection. Chest radiography characteristically shows a picture simulating pulmonary oedema. Bronchoalveolar lavage or lung biopsy demonstrates alveolar secretions that are strongly PAS-positive but contain no organisms and no excessive cellular response. In 30 to 50% of cases the disease improves spontaneously or fails to progress. When intervention is necessary the most effective measure is physical removal of secretions by whole-lung bronchoalveolar lavage. GM-CSF has been given to a few patients, with half seeming to respond. Eradication of infection may be difficult when opportunistic organisms are involved. Survival is about 70% at 10 years.

Chapter.  2111 words.  Illustrated.

Subjects: Respiratory Medicine and Pulmonology

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