Autoimmune Pulmonary Alveolar Proteinosis: “The Sleeping Beauty and the Kissing Drug”

Autoimmune pulmonary alveolar proteinosis (aPAP) the most com-mon form of PAP (90%) is related to the abnormal production of high titers of autoantibodies against the hematopoietic cytokine granulocyte macrophage–colony-stimulating factor (GM-CSF)1. Autoantibodies act neutralizing GM-CSF bioactivity on alveolar macrophages impeding their ability to catabolize phagocytized surfactant as well as their ability to handle microorganisms1. Disease is related to the inappropriate accumulation of surfactant lipoproteins inside alveolar macrophages and diffusely in the airspaces occupying space and exhausting pulmonary functional reserve2. Clinically the disease presents with progressive dyspnea on exertion and hypoxemia that may lead to respiratory insufficiency leading to death2. Occasionally, the slow deteriorating course of the disease may interrupt abruptly due to the occurrence of severe and deadly opportunistic lung infections3. The modern history of PAP begins 20 years ago when two groups o