Title

Update on Diagnosis and Treatment of Adult Pulmonary Alveolar Proteinosis.

Document Type

Article

Publication Date

8-10-2021

Publication Title

Ther Clin Risk Manag

Abstract

Pulmonary alveolar proteinosis (PAP) is a rare pulmonary surfactant homeostasis disorder resulting in buildup of lipo-proteinaceous material within the alveoli. PAP is classified as primary (autoimmune and hereditary), secondary, congenital and unclassifiable type based on the underlying pathogenesis. PAP has an insidious onset and can, in some cases, progress to severe respiratory failure. Diagnosis is often secured with bronchoalveolar lavage in the setting of classic imaging findings. Recent insights into genetic alterations and autoimmune mechanisms have provided newer diagnostics and treatment options. In this review, we discuss the etiopathogenesis, diagnosis and treatment options available and emerging for PAP.

Volume

17

First Page

701

Last Page

710

DOI

10.2147/TCRM.S193884

ISSN

1176-6336

PubMed ID

34408422

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