Universität zu Köln

Schaefer, Stephan C., Funke-Chambour, Manuela and Berezowska, Sabina ORCID: 0000-0001-5442-9791 (2020). Idiopathic pulmonary fibrosis-epidemiology, causes, and clinical course. Pathologe, 41 (1). S. 46 - 52. HEIDELBERG: SPRINGER HEIDELBERG. ISSN 1432-1963

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Abstract

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
Creators:	Creators Email ORCID ORCID Put Code Schaefer, Stephan C. UNSPECIFIED UNSPECIFIED UNSPECIFIED Funke-Chambour, Manuela UNSPECIFIED UNSPECIFIED UNSPECIFIED Berezowska, Sabina UNSPECIFIED orcid.org/0000-0001-5442-9791 UNSPECIFIED
URN:	urn:nbn:de:hbz:38-347376
DOI:	10.1007/s00292-019-00747-x
Journal or Publication Title:	Pathologe
Volume:	41
Number:	1
Page Range:	S. 46 - 52
Date:	2020
Publisher:	SPRINGER HEIDELBERG
Place of Publication:	HEIDELBERG
ISSN:	1432-1963
Language:	German
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language MUC5B PROMOTER POLYMORPHISM; INTERSTITIAL LUNG ABNORMALITIES; RISK; INDIVIDUALS; MORTALITY; DISEASE; COHORT Multiple languages Pathology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/34737