Abstract

Aim: This review intends to recapitulate the knowledge about pulmonary alveolar proteinosis (PAP) and insight into the advances in the pathogenesis and treatment of this condition. Background: A PAP is a seldom occurring disease with numerous possible etiologies. Review results: The disease has an insidious onset and the opinion is mostly delayed due to the fact that patients present late when there is abundant surfactant assimilation in alveoli to diminish gas exchange and cause dyspnea. An appropriate history, typical chest radiographic and high resolution computed tomography (HRCT) findings together with characteristic b ronchoalveolar lavage (BAL) fluid help to elucidate the diagnosis in most cases. However, transbronchial lung biopsy (TBLB) or open lung biopsy may be rarely needed for difficult to diagnose cases. Treatment is needed for patients with symptomatic disease and whole lung lavage is the treatment of choice. Conclusion: A PAP is a rare disease entity with variable natural history. The clinical course varies from respiratory failure to spontaneous resolution. Clinical significance: This review will help to furnish an outline of the various aspects of this disease in light of fresh scientific advancements in the pathogenesis and treatment of this condition. Knowledge about the disease will serve to better define the role of alternative and new therapies for the same.

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