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Systemic sclerosis: Immunopathogenesis, clinical features, and treatment 

Systemic sclerosis: Immunopathogenesis, clinical features, and treatment
Chapter:
Systemic sclerosis: Immunopathogenesis, clinical features, and treatment
Author(s):

Voon H. Ong

and Christopher P. Denton

DOI:
10.1093/med/9780199579655.003.0090
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date: 27 September 2020

Systemic sclerosis (scleroderma, SSc) is a chronic multi-system connective tissue disease of uncertain aetiology with an annual incidence of approximately 10 per million in the UK. With a peak age of onset between 30 and 50 years, SSc has a strong female predominance but the factors that are responsible for this are not apparent. The early hallmarks of SSc are autoimmunity and inflammation with widespread functional and structural microvasculopathy. In advanced disease, end-organ damage occurs as a result of progressive interstitial and vascular fibrosis in the skin and major internal organs. Two major subsets of scleroderma are recognized because of the major differences in the extent of skin involvement and the pattern of internal organ involvement: limited cutaneous systemic sclerosis (lcSSc) in which cutaneous involvement is limited to the hands, face, feet and forearms, and diffuse cutaneous systemic sclerosis (dcSSc) in which there is proximal extension of skin involvement above the elbows and knees and the trunk. A third subset describes a subgroup of patients who demonstrate involvement of the internal organs such as gastrointestinal system or lung fibrosis in the absence of skin disease and this subgroup, scleroderma sine scleroderma, will often present only when end-organ damage is clinically apparent. Although the outcome of SSc has improved considerably over the last decade, the disease carries the highest case fatality among the connective tissue diseases.

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