Timing of Whole Lung Lavage in Autoimmune Pulmonary Alveolar Proteinosis with Concurrent Opportunistic Infection: A...

Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare lung disorder characterized by abnormal accumulation of surfactant in the alveoli, often leading to progressive respiratory failure. While whole lung lavage (WLL) remains the standard therapeutic intervention, its timing and safety in the context of active infection remain a critical clinical dilemma.

We present a fatal case of a 42-year-old Tibetan female with aPAP and progressive respiratory failure requiring V-V ECMO. Critically, serological tests were negative, but targeted next-generation sequencing (tNGS) of bronchoalveolar lavage fluid revealed a dominant mixed opportunistic infection (Cryptococcus neoformans: 3,877 reads; Enterococcus faecium: 118,062 reads). Despite this finding, the patient underwent emergent WLL and subsequently succumbed to the uncontrolled infection. To contextualize our findings, we conducted a systematic literature search in PubMed and Embase from their inception to November 21, 2025, which identified 27 relevant case reports and series. This review demonstrated that the majority of WLL procedures were performed after the active infection was controlled or had resolved following the initiation of antimicrobial therapy. The outcome was generally favorable, with clinical improvement reported in 23 cases (85.2%).

This fatal aPAP case suggests a staged management approach whereby WLL should be deferred until infection control is achieved. Early tNGS-guided pathogen identification appears crucial for directing antimicrobial therapy. This target-then-lavage strategy merits further validation but offers a prudent framework for managing these high-risk patients.