Universität zu Köln

Rosendahl, Ann-Helen, Schoenborn, Katrin and Krieg, Thomas (2022). Pathophysiology of systemic sclerosis (scleroderma). Kaohsiung J. Med. Sci., 38 (3). S. 187 - 196. HOBOKEN: WILEY. ISSN 2410-8650

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Abstract

Systemic sclerosis (scleroderma) is an autoimmune-triggered chronic fibrosing disease that affects the skin and many other organs. Its pathophysiology is complex and involves an early endothelial damage, an inflammatory infiltrate and a resulting fibrotic reaction. Based on a predisposing genetic background, an altered balance of the acquired and the innate immune system leads to the release of many cytokines and chemokines as well as autoantibodies, which induce the activation of fibroblasts with the formation of myofibroblasts and the deposition of a stiff and rigid connective tissue. A curative treatment is still not available but remarkable progress has been made in the management of organ complications. In addition, several breakthroughs in the pathophysiology have led to new therapeutic concepts. Based on these, many new compounds have been developed during the last years, which target these different pathways and offer specific therapeutic approaches.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Rosendahl, Ann-Helen UNSPECIFIED UNSPECIFIED UNSPECIFIED Schoenborn, Katrin UNSPECIFIED UNSPECIFIED UNSPECIFIED Krieg, Thomas UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-662325
DOI:	10.1002/kjm2.12505
Journal or Publication Title:	Kaohsiung J. Med. Sci.
Volume:	38
Number:	3
Page Range:	S. 187 - 196
Date:	2022
Publisher:	WILEY
Place of Publication:	HOBOKEN
ISSN:	2410-8650
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language GROWTH-FACTOR-BETA; GRANULATION-TISSUE; B-CELLS; SKIN FIBROSIS; MAST-CELLS; FIBROBLASTS; APOPTOSIS; AUTOANTIBODIES; RECEPTOR; MYOFIBROBLASTS Multiple languages Medicine, Research & Experimental Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/66232