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.
Footnotes
↵* The author has indicated that he has received grant or research support from the Immunex Corporation for research on GM-CSF.
- Copyright © 2001 The Cleveland Clinic Foundation. All Rights Reserved.
- Department of Pulmonary and Critical Care Medicine, Cleveland Clinic
- Department of Pulmonary and Critical Care Medicine, Cleveland Clinic
- Department of Pulmonary and Critical Care Medicine, Cleveland Clinic
- ADDRESS:
Mani Kavuru, MD, Department of Pulmonary and Critical Care Medicine, A90, The Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH 44195; e-mail kavurum{at}ccf.org.
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.
Footnotes
↵* The author has indicated that he has received grant or research support from the Immunex Corporation for research on GM-CSF.
- Copyright © 2001 The Cleveland Clinic Foundation. All Rights Reserved.