Universität zu Köln

Kasperkiewicz, Michael, Eming, Ruediger, Behzad, Melika, Hunzelmann, Nicolas, Meurer, Michael, Schulze-Koops, Hendrik, von Wussow, Peter, Hertl, Michael, Zillikens, Detlef, Freivogel, Klaus, Doerner, Thomas and Schmidt, Enno ORCID: 0000-0002-1206-8913 (2012). Systemic sclerosis - dermatological aspects. Part 1: Pathogenesis, epidemiology, clinical findings. J. Dtsch. Dermatol. Ges., 10 (10). S. 727 - 733. HOBOKEN: WILEY-BLACKWELL. ISSN 1610-0379

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Abstract

Systemic sclerosis is a chronic inflammatory multiorgan disease belonging to the group of collagen-vascular disorders. With a prevalence of 10/100,000 inhabitants it may be regarded a rather rare disease. Its etiology and pathogenesis have still not been elucidated in detail, especially with regard to the differential involvement of skin and the cause of the clinically heterogeneous disease courses. Various components of the vasculature, connective tissue as well as the immune system are involved in a yet unknown sequence and significance. Patients need to be cared for in an interdisciplinary fashion depending on the individual organ involvement. Apart from the skin, the heart, kidneys and lungs are mainly affected in addition to frequent gastrointestinal and musculoskeletal symptoms. Clinically two distinct subsets may be separated, acral (also termed limited) and diffuse scleroderma, which are characterized by anti-centromere and anti-Scl-70/topoisomerase-1 antibodies, respectively. Recent data demonstrate a poor prognosis even in limited disease when pulmonary arterial hypertension develops at an early stage. In diffuse disease sudden and rapid onset will result in a sclerosis of major internal organs and early death in many cases.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Kasperkiewicz, Michael UNSPECIFIED UNSPECIFIED UNSPECIFIED Eming, Ruediger UNSPECIFIED UNSPECIFIED UNSPECIFIED Behzad, Melika UNSPECIFIED UNSPECIFIED UNSPECIFIED Hunzelmann, Nicolas UNSPECIFIED UNSPECIFIED UNSPECIFIED Meurer, Michael UNSPECIFIED UNSPECIFIED UNSPECIFIED Schulze-Koops, Hendrik UNSPECIFIED UNSPECIFIED UNSPECIFIED von Wussow, Peter UNSPECIFIED UNSPECIFIED UNSPECIFIED Hertl, Michael UNSPECIFIED UNSPECIFIED UNSPECIFIED Zillikens, Detlef UNSPECIFIED UNSPECIFIED UNSPECIFIED Freivogel, Klaus UNSPECIFIED UNSPECIFIED UNSPECIFIED Doerner, Thomas UNSPECIFIED UNSPECIFIED UNSPECIFIED Schmidt, Enno UNSPECIFIED orcid.org/0000-0002-1206-8913 UNSPECIFIED
URN:	urn:nbn:de:hbz:38-482453
DOI:	10.1111/j.1610-0387.2012.07931.x
Journal or Publication Title:	J. Dtsch. Dermatol. Ges.
Volume:	10
Number:	10
Page Range:	S. 727 - 733
Date:	2012
Publisher:	WILEY-BLACKWELL
Place of Publication:	HOBOKEN
ISSN:	1610-0379
Language:	German
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language Dermatology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/48245