Mahsa Mirdamadi is professor of Pulmonary Medicine, Iran University of Medical Sciences, Rajaie Cardiovascular Medical and Research center of Medical Sciences, Tehran
Abstract
Pulmonary Alveolar Proteinosis PAP is a rare pulmonary disease characterized by alveolar accumulation of surfactant. The Primary PAP accounts for approximately 90% of all cases and appears to be mediated through a circulating neutralizing antibody (anti granulocyte macrophage colony stimulating factor and immunoglobulin neutralizing immunoglobulin G antibody).
The reported incidence is 1:300,000 persons in the United States. Pulmonary Alveolar Proteinosis occurs in 3 clinically distinct forms: congenital, secondary and acquired. Acquired Pulmonary Alveolar Proteinosis may be due to inhalational exposure, hematologic cancers, immune deficiency or medications.