PRIMARY PULMONARY ALVEOLAR PROTEINOSIS: A CASE REPORT AND A REVIEW OF THE LITERATURE

Rosen et al. first described pulmonary alveolar proteinosis (PAP) in 1958. PAP is characterized by the accumulation of phospholipoproteinaceous material in alveolar spaces. The prevalence of PAP is difficult to determine from the literature, however, it is rare. The etiology of this disorder is not yet known. There are two types of PAP based on associated disorders: primary (idiopathic) type, in the absence of identifiable coexisting abnormalities, and secondary type, when conditions including infection, malignant hematologic disease and inorganic dust exposure coexist. In Saudi Arabia there is only one previous report of PAP in a Saudi pregnant female who presented with respiratory failure, and was found to have pulmonary Mycobacterium tuberculosis infection, i.e., the secondary form of PAP. In this communication, we report the first adult Saudi male patient with primary pulmonary alveolar proteinosis, followed by an updated literature review of disease etiology, clinical manifestations, diagnosis, natural history, and treatment.