Development of a clinical method to assess disease severity and treatment effect in idiopathic pulmonary alveolar proteinosis

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Autoantibodies against granulocyte-marcophage colony-stimulating factor (GM-CSF) are consistently and specifically detected in the serum and bronchoalveolar lavage fluid (BALF) in the patients with idiopathic pulmonary alveolar proteinosis (iPAP). The concentration of the autoantibody is 81.9 (median, range 4.7-686.3) μg/ml in the serum and 1.15 (0.09-5.4) μg/ml in the BALF.Autoantibodies against GMCSF have very high neutralizing activity and binding avidity, binding specificity. They recognize GMCSF superstructure. In iPAP patients, the autoantibodies against GM-CSF is proved to have enough capability to abrogate GM-CSF bioactivity in the lung. These results are published in the American Journal of Hematology.Significant correlations are observed between those parameters which indicate disease severity (i.e. PaO2, A-aDO2, and DLCO) and serum biochemical parameters (i.e. CEA, KL-6, SP-A). However, serum and BALF concentrationsof the autoantibody and neutralizing activity of the autoantibody purified from the serum have no correlation with disease severity.In 2 of 4 patients, the values of GM-CSF neutralizing activity in the serum paralleled the disease severity. This parameter has the possibility to be highly disease specific and minimum-invasive parameters for estimating disease severity of iPAP.