[Idiopathic pulmonary fibrosis-epidemiology, causes, and clinical course].

Schäfer, Stephan C.; Funke-Chambour, Manuela; Berezowska, Sabina (2020). [Idiopathic pulmonary fibrosis-epidemiology, causes, and clinical course]. Der Pathologe, 41(1), pp. 46-51. Springer 10.1007/s00292-019-00747-x

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Idiopathic pulmonary fibrosis (IPF) plays a special role within the group of interstitial lung diseases (ILDs) due to its inexorable progression and its specific medical treatment. With a median survival of only 2-3 years from the time of diagnosis, the prognosis is worse than many carcinomas.In contrast to other ILDs, IPF does not respond to anti-inflammatory treatment with corticosteroids but rather demands a specific medical therapy. Even though this cannot cure the disease, it can prolong survival. Lung transplantation is the only cure for progressive lung fibrosis. The clinical course is individual and difficult to predict. Acute exacerbations accelerate the clinical course and lead to high mortality.The underlying pathomechanisms of IPF, with its complex immunological and inflammatory processes and external impacts, have been the focus of recent research. Lifestyle and environmental influences are held responsible for much of its natural history. Smoking, pneumotoxic medications, and inhalation of dusts are well-known risk factors. Likewise, genetic and hereditary factors play a crucial role.This short review focuses on the peculiarities of IPF within the group of ILDs, especially in relation to its underlying mechanisms and clinical progression.

Item Type:

Journal Article (Further Contribution)


04 Faculty of Medicine > Department of Gastro-intestinal, Liver and Lung Disorders (DMLL) > Clinic of Pneumology
04 Faculty of Medicine > Service Sector > Institute of Pathology > Clinical Pathology
04 Faculty of Medicine > Service Sector > Institute of Pathology

UniBE Contributor:

Funke-Chambour, Manuela, Berezowska, Sabina Anna


500 Science > 570 Life sciences; biology
600 Technology > 610 Medicine & health








Heidi Lobsiger

Date Deposited:

24 Feb 2020 15:00

Last Modified:

05 Dec 2022 15:36

Publisher DOI:


PubMed ID:


Uncontrolled Keywords:

Epidemiology Familial lung fibrosis Genetic factors Interstitial lung disease Pathogenesis





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