The natural history of progressive fibrosing interstitial lung diseases
The result's identifiers
Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00064190%3A_____%2F19%3AN0000071" target="_blank" >RIV/00064190:_____/19:N0000071 - isvavai.cz</a>
Result on the web
<a href="http://dx.doi.org/10.1186/s12931-019-1022-1" target="_blank" >http://dx.doi.org/10.1186/s12931-019-1022-1</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.1186/s12931-019-1022-1" target="_blank" >10.1186/s12931-019-1022-1</a>
Alternative languages
Result language
angličtina
Original language name
The natural history of progressive fibrosing interstitial lung diseases
Original language description
A proportion of patients with certain types of interstitial lung disease (ILD), including chronic hypersensitivity pneumonitis and ILDs associated with autoimmune diseases, develop a progressive fibrosing phenotype that shows similarities in clinical course to idiopathic pulmonary fibrosis. Irrespective of the clinical diagnosis, these progressive fibrosing ILDs show commonalities in the underlying pathogenetic mechanisms that drive a self-sustaining process of pulmonary fibrosis. The natural history of progressive fibrosing ILDs is characterized by decline in lung function, worsening of symptoms and health-related quality of life, and early mortality. Greater impairment in forced vital capacity or diffusion capacity of the lungs for carbon monoxide, and a greater extent of fibrotic changes on a computed tomography scan, are predictors of mortality in patients with fibrosing ILDs. However, the course of these diseases is heterogenous and cannot accurately be predicted for an individual patient. Data from ongoing clinical trials and patient registries will provide a better understanding of the clinical course and impact of progressive fibrosing ILDs.
Czech name
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Czech description
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Classification
Type
J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database
CEP classification
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OECD FORD branch
30203 - Respiratory systems
Result continuities
Project
—
Continuities
N - Vyzkumna aktivita podporovana z neverejnych zdroju
Others
Publication year
2019
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů
Data specific for result type
Name of the periodical
RESPIRATORY RESEARCH
ISSN
1465-993X
e-ISSN
1465-9921
Volume of the periodical
20
Issue of the periodical within the volume
03.2019
Country of publishing house
GB - UNITED KINGDOM
Number of pages
8
Pages from-to
Article Number: 57
UT code for WoS article
000461673500001
EID of the result in the Scopus database
2-s2.0-85063006773