GM-CSF pathway correction in pulmonary alveolar proteinosis.
BACKGROUND
Pulmonary alveolar proteinosis (PAP) is a rare disease in which the abnormalities in surfactant metabolism are caused most often by impairments of GM-CSF pathway at different levels in different disease subsets (congenital, secondary, acquired/idiopathic) and for which there are only few, costly invasive therapeutic methods.


METHODS
This review discusses these impairments, and their pathogenic and clinical consequences along with potential corrective therapies such as exogenous inhaled GM-CSF.


RESULTS
Among the PAP disease subsets, in autoimmune PAP the GM-CSF autoantibodies play a major role in disease pathogenesis and their deleterious pulmonary effects can be blocked efficaciously with inhaled GM-CSF.


CONCLUSIONS
In PAP correction of the abnormalities of the GM-CSF pathway represent a plausible approach demonstrated to be efficacious also in the case of inhaled GM-CSF used for autoimmune PAP.
Antoniu
Sabina A
SA
University of Medicine and Pharmacy Gr T Popa Iasi, Pulmonary Disease University Hospital, Department of Internal Medicine II- Pulmonary Disease, 30 Dr I Cihac Str, 700115 Iasi, Romania. sabina.antonela.antoniu@pneum.umfiasi.ro
D000280:Administration, Inhalation; D000818:Animals; D001323:Autoantibodies / Q000378:metabolism; D016178:Granulocyte-Macrophage Colony-Stimulating Factor / Q000008:administration & dosage* / Q000276:immunology / Q000378:metabolism; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage*; D011649:Pulmonary Alveolar Proteinosis / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology; D015398:Signal Transduction / Q000187:drug effects*; D016896:Treatment Outcome
D001323:Autoantibodies; D007155:Immunologic Factors; D016178:Granulocyte-Macrophage Colony-Stimulating Factor