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Systemic sclerosis 

Systemic sclerosis
Systemic sclerosis

Christopher P. Denton

and Carol M. Black


November 30, 2011: This chapter has been re-evaluated and remains up-to-date. No changes have been necessary.

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date: 19 September 2021

The scleroderma spectrum of disorders includes a number of diseases that have Raynaud’s phenomenon or skin sclerosis in common, comprising (1) limited cutaneous scleroderma; (2) systemic sclerosis (SSc)—the most important form of scleroderma—limited cutaneous SSc, diffuse cutaneous SSc, and overlap syndromes (with features of another autoimmune rheumatic disease, e.g. systemic lupus erythematosus); (3) Raynaud’s phenomenon—autoimmune (with antinuclear or other SSc-associated antibodies) or primary. These conditions affect women four times as often as men, most often beginning in the fifth decade....

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