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Autoimmune PAP

Whole lung lavage remains central to PAP treatment amid calls for standardized guidelines

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Whole lung lavage (WLL) remains the cornerstone treatment for pulmonary alveolar proteinosis (PAP), despite advancements in understanding the disease and the development of GM-CSF-based therapies, according to a new review. A critical need however remains for standardized protocols and international guidelines to optimize and unify PAP management practices.

Key Takeaways:

  • Pathophysiology: Autoimmune PAP, the most common form, results from antigranulocyte-macrophage colony-stimulating factor (GM-CSF) autoantibodies, leading to GM-CSF deficiency and impaired surfactant clearance.
  • Treatment Landscape: Although emerging GM-CSF-based therapies show promise, WLL continues to be the primary therapeutic approach due to the lack of international treatment guidelines or standardized protocols.
  • Technique Evolution: Advances in WLL emphasize preprocedural planning, patient assessment, anesthetic approaches, and postprocedural care to optimize outcomes. Sequential bronchoscopic lavage offers a minimally invasive alternative in select cases.

Despite progress, the review underscores the need for global consensus on PAP management and further research to refine and standardize WLL protocols.

Reference
Tay CK, Kumar A, Hsu AAL, et al. Whole lung and sequential bronchoscopic lavage for pulmonary alveolar proteinosis. Curr Opin Pulm Med. 2025;31(1):41-52. doi: 10.1097/MCP.0000000000001138. Epub 2024 Nov 21. PMID: 39569652.

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