Spontaneous Resolution in Autoimmune Pulmonary Alveolar Proteinosis: A Case Series.

Abstract

Pulmonary alveolar proteinosis (PAP) is a rare lung condition characterized by the accumulation of proteinaceous material within the alveoli. The acquired form is often autoimmune, driven by autoantibodies against granulocyte-macrophage colony-stimulating factor. Presentation of autoimmune PAP may range from incidental detection in asymptomatic patients to advanced respiratory failure. Treatment typically involves whole lung lavage or granulocyte-macrophage colony-stimulating factor therapy in symptomatic patients; spontaneous resolution is rare in severe cases. Here, we report 3 cases of autoimmune PAP who presented with resting hypoxia and exhibited spontaneous resolution without significant treatment. All 3 cases had a history of occupational inhalational exposure to noxious gases, and they improved following sustained cessation of exposure. We hypothesize that occupational or environmental noxious inhalation exposure may have a role in disease expression in some cases of autoimmune PAP, and such cases may show spontaneous resolution following cessation of the offending exposure.