Whole lung and sequential bronchoscopic lavage for pulmonary alveolar proteinosis.

Abstract

Purpose of review: Pulmonary alveolar proteinosis (PAP) is a diffuse lung disease that results from the accumulation of lipoproteinaceous material in the alveoli due to abnormal surfactant homeostasis. Since its introduction in the 1960s, whole lung lavage (WLL) has been the primary treatment for PAP. This review focuses on WLL, including its technique modifications, and sequential bronchoscopic lavage.

Recent findings: Autoimmune PAP, which accounts for the majority of cases, occurs when antigranulocyte-macrophage colony-stimulating factor (GM-CSF) autoantibodies lead to the deficiency of bioavailable GM-CSF. At present, there are no international guidelines or consensus statements for PAP treatment. Traditionally, therapeutic decisions are made based on the severity and type of PAP. Despite emerging data on GM-CSF-based therapies, WLL remains a central component in the therapeutic strategy for PAP.

Summary: Although the technique of WLL has evolved over time, there is still no universally adopted, standardized protocol. However, key periprocedural aspects - such as preprocedural planning, patient evaluation, anesthetic technique, lavage protocol, and postprocedural care - remain essential to ensuring the safety and success of WLL.