Pulmonary Alveolar Proteinosis (PAP) is a rare syndrome characterized by pulmonary surfactant accumulation within\r\nthe alveolar spaces. It occurs with a reported prevalence of 0.1 per 100,000 individuals and in distinct clinical forms:\r\nautoimmune (previously referred to as the idiopathic form, represents the vast majority of PAP cases, and is\r\nassociated with Granulocyte-Macrophage Colony Stimulating Factor (GM-CSF) auto-antibodies; GMAbs), secondary\r\n(is a consequence of underlying disorders), congenital (caused by mutations in the genes encoding for the GM-CSF\r\nreceptor), and PAP-like syndromes (disorders associated with surfactant gene mutations). The clinical course of PAP\r\nis variable, ranging from spontaneous remission to respiratory failure. Whole lung lavage (WLL) is the current\r\nstandard treatment for PAP patients and although it is effective in the majority of cases, disease persistence is not\r\nan unusual outcome, even if disease is well controlled by WLL.\r\nIn this paper we review the therapeutic strategies which have been proposed for the treatment of PAP patients and\r\nthe progress which has been made in the understanding of the disease pathogenesis
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