PT - JOURNAL ARTICLE AU - Peter Mazzone AU - Mary Jane Thomassen AU - Mani Kavuru TI - Our new understanding of pulmonary alveolar proteinosis: What an internist needs to know DP - 2001 Dec 01 TA - Cleveland Clinic Journal of Medicine PG - 977--993 VI - 68 IP - 12 4099 - http://www.ccjm.org/content/68/12/977.short 4100 - http://www.ccjm.org/content/68/12/977.full SO - Cleve Clin J Med2001 Dec 01; 68 AB - 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.