ABSTRACT

Alveolar proteinosis is a rare disease with an estimated incidence of 0.36 cases per million, male to female ratio is 2.1: 1, with a median age of 51 years old. Among the important antecedents are smoking and exposure to inorganic dust. It is characterized by abnormal accumulation of proteinaceous material in the alveolar spaces with impaired gas exchange. Ranked in congenital, primary or secondary. The diagnosis is suspected on clinical and radiological findings characterized by alveolar attenuation scattered areas in ground -glass. Respiratory function tests suggest restrictive pattern. The diagnostic and therapeutic bronchoscopy is finding milky material, opaque and frothy. Lung biopsy may be useful in some cases. There are other treatments which are not yet standardized. We present 2 cases of alveolar proteinosis and its clinical evolution.

KEYWORDS

Granulocyte-macrophage colony-stimulating factor, pulmonary alveolar proteinosis, surfactant metabolism dysfunction.

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