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Published on: June 23, 2019
Our new understanding of pulmonary alveolar proteinosis: what an internist needs to know
P Mazzone1, M J Thomassen, M Kavuru
1Department of Pulmonary and Critical Care Medicine, Cleveland Clinic Foundation, OH 44195, USA.
Abstract:
Pulmonary alveolar proteinosis (PAP; the accumulation of surfactant lipids and proteins in the alveoli) has a number of infectious and environmental causes but is usually idiopathic. The clinical presentation of PAP is nonspecific; thus, the diagnosis is frequently missed, leading to inappropriate therapy and unnecessary morbidity. Recent advances suggest that a deficiency in granulocyte-macrophage colony-stimulating factor (GM-CSF) activity may lead to this surfactant accumulation. Anti-GM-CSF antibodies have been found in PAP patients, fueling speculation that PAP may be an autoimmune disease. These findings are being translated into novel forms of therapy.
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