Our new understanding of pulmonary alveolar proteinosis: What an internistneeds to know

Citation
P. Mazzone et al., Our new understanding of pulmonary alveolar proteinosis: What an internistneeds to know, CLEV CLIN J, 68(12), 2001, pp. 977
Citations number
51
Categorie Soggetti
General & Internal Medicine
Journal title
CLEVELAND CLINIC JOURNAL OF MEDICINE
ISSN journal
08911150 → ACNP
Volume
68
Issue
12
Year of publication
2001
Database
ISI
SICI code
0891-1150(200112)68:12<977:ONUOPA>2.0.ZU;2-J
Abstract
Pulmonary alveolar proteinosis (PAP; the accumulation of surfactant lipids and proteins in the alveoli) has a number of infectious and environmental c auses but is usually idiopathic. The clinical presentation of PAP is nonspe cific; thus, the diagnosis is frequently missed, leading to inappropriate t herapy 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 foun d in PAP patients, fueling speculation that PAP may be an autoimmune diseas e. These findings are being translated into novel forms of therapy.