Outpatient selected lobar lavage: A novel approach to managing pulmonary alveolar proteinosis

Abstract

BACKGROUND Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by the accumulation of surfactant components in alveoli, impairing gas exchange. The mainstay of treatment remains whole lung lavage (WLL); however, selected lobar lavage (SLL) using smaller volumes of normal saline has also been described. Due to the marked variability in the severity and natural history of PAP, there is a need for more tailored treatment approaches. METHODS Repeated outpatient SLL were performed using conscious sedation and fiberoptic bronchoscopy at intervals between 1 and 6 weeks. Each procedure targeted a single lobe, with the order of lavages based on degree of radiographic involvement. The bronchoscope was modified with a flushing pump to deliver warm normal saline in 100 mL aliquots up to a maximum of 3000 mL. Computed tomography scans and pulmonary function testing were performed at baseline and repeated after completion of all lavages. RESULTS Radiographic and symptomatic improvement was noted in all cases and physiologic improvement was noted in three of four cases. The only complication was transient procedural hypoxemia. All patients were able to be discharged home on the same day of their procedure. INTERPRETATION Our experience suggests outpatient SLL is a safe and effective alternative to WLL in mild-moderate cases of PAP with the benefits of avoiding admission to hospital, general anesthesia, and endotracheal intubation with single-lung ventilation. All patients remained relapse free for a minimum follow-up duration of 11 months.