Whole Lung Lavage in Pulmonary Alveolar Proteinosis

Abstract

Pulmonary alveolar proteinosis (PAP) is a rare diffuse lung syndrome characterized by impaired surfactant clearance and alveolar filling, most commonly the result of autoimmune neutralization of granulocyte-macrophage colony-stimulating factor (GM-CSF) by autoantibodies. Whole lung lavage (WLL) remains the cornerstone of treatment, yet procedural practices vary widely across institutions. This report presents a reproducible, evidence-aligned protocol for WLL developed across 2 high-volume centers, detailing perioperative management, technical execution, and follow-up. We describe our approach to patient selection, contraindication screening, anesthesia and airway strategy, and the stepwise lavage process using warmed saline instillation and drainage under general anesthesia with single-lung ventilation. Lavage is performed in 2 staged sessions, guided by effluent clarity and clinical tolerance. Intraoperative challenges such as hypoxemia, fluid spillover, or poor return are anticipated and addressed using structured response algorithms. Postprocedural care includes diuresis, lung re-expansion measures, and early mobilization, with discharge typically within 24 to 48 hours for elective outpatients. Most patients experience rapid improvement in symptoms, gas exchange, and functional capacity; however, recurrence is common, with one-third of patients requiring repeat lavage within 2 to 3 years. Inhaled GM-CSF therapy now is considered after WLL in all eligible patients, especially those with incomplete response or high relapse risk, prolonging remission and reducing the need for subsequent procedures. Our experience supports a combined strategy of lavage, adjunctive therapy, and longitudinal surveillance to achieve sustained disease control. By emphasizing multidisciplinary coordination, individualized risk assessment, and protocolized execution, this framework aims to enhance safety, reproducibility, and long-term monitoring to reduce recurrence and improve outcomes in PAP.

Keywords: double lumen tube; granulocyte-macrophage colony-stimulating factor; pulmonary alveolar proteinosis; whole lung lavage.