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16-05-2024 | REVIEW

Autoimmune Pulmonary Alveolar Proteinosis: A Review of Pathogenesis and Emerging Therapies

Authors: Akshay Mathavan, Akash Mathavan, Swaminathan Perinkulam Sathyanarayanan, Cormac McCarthy, Ali Ataya

Published in: Current Pulmonology Reports | Issue 3/2024

Abstract

Purpose of Review

Autoimmune pulmonary alveolar proteinosis is a heterogenous clinical syndrome of disordered surfactant clearance due to a dysfunctional granulocyte–macrophage colony-stimulating factor signaling axis in the setting of polyclonal autoantibody generation. Recent advancements identifying key mechanistic drivers of this disease have been made. This clinical review summarizes current knowledge of autoimmune pulmonary alveolar proteinosis with an emphasis on contemporary findings on pathogenesis and emerging therapies.

Recent Findings

A disturbed granulocyte–macrophage colony-stimulating factor signaling axis leads to downstream dysregulation of cholesterol export within alveolar macrophages. Accumulation of cholesterol impedes surfactant clearance and propagates the syndrome’s disease process.

Summary

Whole lung lavage therapy is an invasive procedure performed under general anesthesia which remains the standard of care for autoimmune pulmonary alveolar proteinosis. Augmentation of the defective signaling axis with recombinant human granulocyte-macrophage colony-stimulating factor is a promising treatment modality.

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Title: Autoimmune Pulmonary Alveolar Proteinosis: A Review of Pathogenesis and Emerging Therapies
Authors: Akshay Mathavan
Akash Mathavan
Swaminathan Perinkulam Sathyanarayanan
Cormac McCarthy
Ali Ataya
Publication date: 16-05-2024
Publisher: Springer US
Published in: Current Pulmonology Reports / Issue 3/2024
Electronic ISSN: 2199-2428
DOI: https://doi.org/10.1007/s13665-024-00356-x

PD Dr. Carsten W. Israel

Developed by: Springer Medizin

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