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Pulmonary alveolar proteinosis 

Pulmonary alveolar proteinosis
Chapter:
Pulmonary alveolar proteinosis
Author(s):

S. J. Bourke

DOI:
10.1093/med/9780198746690.003.0427
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date: 07 March 2021

In 90% of cases pulmonary alveolar proteinosis is caused by autoimmune antibodies to GM-CSF which impair the function of alveolar macrophages in clearing surfactant from the alveoli, giving rise to impaired gas exchange, breathlessness, and respiratory failure. Chest radiography shows extensive alveolar shadowing simulating pulmonary oedema, and CT scanning shows a characteristic ‘crazy paving’ pattern. The presence of GM-CSF antibodies in the serum is useful in diagnosis. Bronchoalveolar lavage or lung biopsy demonstrates alveolar secretions that are strongly PAS-positive. Treatment is by physical removal of the lipoproteinaceous material from the alveoli by whole-lung lavage. Massive inhalation of dust and fumes may overwhelm macrophage function, giving rise to secondary pulmonary alveolar proteinosis.

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