Universität zu Köln

Jerjen, Rebekka, Nikpour, Mandana, Krieg, Thomas, Denton, Christopher P. and Saracino, Amanda M. (2022). Systemic sclerosis in adults. Part I: Clinical features and pathogenesis. J. Am. Acad. Dermatol., 87 (5). S. 937 - 955. NEW YORK: MOSBY-ELSEVIER. ISSN 1097-6787

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Abstract

Systemic sclerosis (SSc), also referred to as systemic scleroderma or scleroderma, is a rare, complex immune-mediated connective tissue disease characterized by progressive skin fibrosis and other clinically heterogenous features. The etiopathogenesis of SSc involves vasculopathy and immune system dysregulation occurring on a permissive genetic and epigenetic background, ultimately leading to fibrosis. Recent developments in our understanding of disease-specific autoantibodies and bioinformatic analyses has led to a reconsideration of the purely clinical classification of diffuse and limited cutaneous SSc subgroups. Autoantibody profiles are predictive of skin and internal organ involvement and disease course. Early diagnosis of SSc, with commencement of disease-modifying treatment, has the potential to improve patient outcomes. In SSc, many of the clinical manifestations that present early signs of disease progression and activity are cutaneous, meaning dermatologists can and should play a key role in the diagnosis and management of this significant condition. The first article in this continuing medical education series discusses the epidemiology, clinical characteristics, and pathogenesis of SSc in adults, with an emphasis on skin manifestations, the important role of dermatologists in recognizing these, and their correlation with systemic features and disease course.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Jerjen, Rebekka UNSPECIFIED UNSPECIFIED UNSPECIFIED Nikpour, Mandana UNSPECIFIED UNSPECIFIED UNSPECIFIED Krieg, Thomas UNSPECIFIED UNSPECIFIED UNSPECIFIED Denton, Christopher P. UNSPECIFIED UNSPECIFIED UNSPECIFIED Saracino, Amanda M. UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-687368
DOI:	10.1016/j.jaad.2021.10.065
Journal or Publication Title:	J. Am. Acad. Dermatol.
Volume:	87
Number:	5
Page Range:	S. 937 - 955
Date:	2022
Publisher:	MOSBY-ELSEVIER
Place of Publication:	NEW YORK
ISSN:	1097-6787
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language EULAR SCLERODERMA TRIALS; ENVIRONMENTAL RISK-FACTORS; POLYMERASE-III ANTIBODIES; ISCHEMIC DIGITAL ULCERS; ORGAN INVOLVEMENT; RENAL CRISIS; CLASSIFICATION CRITERIA; NAILFOLD CAPILLAROSCOPY; ANTICENTROMERE ANTIBODY; RESEARCH COHORT Multiple languages Dermatology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/68736