Pulmonary alveolar proteinosis: Clinical and morphological overview of a rare disease associated with macrophage dysfunction

Result code in IS VaVaI

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Result on the web

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DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.4149/gpb_2024038" target="_blank" >10.4149/gpb_2024038</a>

Result language

angličtina

Original language name

Pulmonary alveolar proteinosis: Clinical and morphological overview of a rare disease associated with macrophage dysfunction

Original language description

Pulmonary alveolar proteinosis (PAP) is a rare disease characterised by excessive accumulation of surfactant components in alveolar macrophages, alveoli, and peripheral airways. The accumulation of surfactant is associated with only a minimal inflammatory response but can lead to the development of pulmonary fibrosis. Three clinical forms of PAP are distinguished - primary, secondary and congenital. In recent years, significant findings have helped to clarify the ethiology and pathogenesis of the disease. Apart from impaired surfactant protein function, a key role in the development of PAP is played by signal pathway of granulocyte and macrophage colonies stimulating growth factor (GM-CSF) which is necessary for the functioning of alveolar macrophages and for surfactant homeostasis. Surfactant is partially degraded by alveolar macrophages that are stimulated by GM-CSF. The role of GM-CSF has been shown especially in primary PAP, which is currently considered an autoimmune disease involving the development of GM-CSF neutralising autoantibodies. Clinically, the disease may be silent or manifest with dyspnoeic symptoms triggered by exertion and cough. However, there is a 10 to 15% rate of patients who develop respiratory failure. Total pulmonary lavage is regarded as the standard method of treatment. In addition, recombinant human GM-CSF has been studied as a prospective therapy for the treatment of PAP.

Czech name

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Czech description

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Type

J_imp - Article in a specialist periodical, which is included in the Web of Science database

CEP classification

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OECD FORD branch

30109 - Pathology

Project

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Continuities

V - Vyzkumna aktivita podporovana z jinych verejnych zdroju

Publication year

2025

Confidentiality

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Name of the periodical

General physiology and biophysics

ISSN

0231-5882

e-ISSN

1338-4325

Volume of the periodical

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Issue of the periodical within the volume

1

Country of publishing house

SK - SLOVAKIA

Number of pages

11

Pages from-to

1-11

UT code for WoS article

001439867000001

EID of the result in the Scopus database

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