Treatment of Adult Primary Alveolar Proteinosis.

Abstract:

:Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by the accumulation of surfactant-like lipoproteinaceous material in the distal air spaces and terminal bronchi, which may lead to impaired gas exchange. This accumulation of surfactant is due to decreased clearance by the alveolar macrophages. Its primary, most common form, is currently considered an autoimmune disease. Better knowledge of the causes of PAP have led to the emergence of alternatives to whole lung lavage, although this is still considered the treatment of choice. Most studies are case series, often with limited patient numbers, so the level of evidence is low. Since the severity of presentation and clinical course are variable, not all patients will require treatment. Due to the low level of evidence, some objective criteria based on expert opinion have been arbitrarily proposed in an attempt to define in which patients it is best to initiate treatment.

journal_name

Arch Bronconeumol

authors

Rodríguez Portal JA

doi

10.1016/j.arbres.2015.02.003

subject

Has Abstract

pub_date

2015-07-01 00:00:00

pages

344-9

issue

7

eissn

0300-2896

issn

1579-2129

pii

S0300-2896(15)00075-7

journal_volume

51

pub_type

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