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:
CreatorsEmailORCIDORCID Put Code
Kasperkiewicz, MichaelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Eming, RuedigerUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Behzad, MelikaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hunzelmann, NicolasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Meurer, MichaelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schulze-Koops, HendrikUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
von Wussow, PeterUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hertl, MichaelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Zillikens, DetlefUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Freivogel, KlausUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Doerner, ThomasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schmidt, EnnoUNSPECIFIEDorcid.org/0000-0002-1206-8913UNSPECIFIED
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:
KeywordsLanguage
DermatologyMultiple languages
URI: http://kups.ub.uni-koeln.de/id/eprint/48245

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