Abstract

Autoimmune (primary) pulmonary alveolar proteinosis (aPAP) is a rare autoimmune lung disease characterized by accumulation of surfactant-derived lipoproteinaceous material in the alveoli, caused by dysfunction of alveolar macrophages due to the deficiency of granulocyte-macrophage colony-stimulating factor (GM-CSF) after neutralization by anti-GM-CSF autoantibodies. In this article, we will analyse two cases of the disease and provide an overview of the nature of the disease, its ology, diagnostics, and related treatments, including Estonia’s first whole lung lavage. The first patient was a 51-year-old man at the time of diagnosis, whose first-line treatment for aPAP (whole lung lavage) was ineffective. Plasma exchange was also of no benefit. Only the initiation of rituximab treatment was effective, after which the disease has remained in remission. In the second case, aPAP was diagnosed in a 42-year-old man, who also received treatment with rituximab, achieving an initial positive effect. However, the patient discontinued therapy and subsequently developed severe Klebsiella pneumoniae-induced pneumonia, complicated by sepsis and multiple organ failure. Despite intensive care (including the application of extracorporeal membrane oxygenation), the patient died.