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Pulmonary alveolar proteinosis 

Pulmonary alveolar proteinosis

Chapter:
Pulmonary alveolar proteinosis
Author(s):

D.J. Hendrick

and S.J. Bourke

DOI:
10.1093/med/9780199204854.003.181407_update_002

July 30, 2015: This chapter has been re-evaluated and remains up-to-date. No changes have been necessary.

Update:

Diagnosis—utility of autoantibodies against GM-CSF.

Updated on 27 Feb 2014. The previous version of this content can be found here.
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date: 18 August 2017

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. Computed tomography shows a characteristic ‘crazy paving’ pattern and the demonstration of anti-GM CSF antibodies in the serum is useful in diagnosis. 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....

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