Our new understanding of pulmonary alveolar proteinosis: what an internist needs to know.
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.
Mazzone
P
P
Department of Pulmonary and Critical Care Medicine, Cleveland Clinic Foundation, OH 44195, USA.
Thomassen
M J
MJ
Kavuru
M
M
D000293:Adolescent; D000328:Adult; D000368:Aged; D000818:Animals; D001323:Autoantibodies / Q000032:analysis; D016026:Bone Marrow Transplantation; D001992:Bronchoalveolar Lavage Fluid; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D016178:Granulocyte-Macrophage Colony-Stimulating Factor / Q000008:administration & dosage / Q000235:genetics / Q000276:immunology / Q000627:therapeutic use; D006801:Humans; D007231:Infant, Newborn; D008168:Lung / Q000473:pathology; D008297:Male; D051379:Mice; D018345:Mice, Knockout; D008875:Middle Aged; D011649:Pulmonary Alveolar Proteinosis / Q000175:diagnosis / Q000209:etiology / Q000473:pathology / Q000628:therapy; D013902:Radiography, Thoracic; D012129:Respiratory Function Tests; D007507:Therapeutic Irrigation; D013997:Time Factors; D014057:Tomography, X-Ray Computed
D001323:Autoantibodies; D016178:Granulocyte-Macrophage Colony-Stimulating Factor