Abstract
Systemic sclerosis (SSc) or scleroderma is an autoimmune connective tissue disease of as-yet unknown etiology. Clinically, SSc is one of a group of illnesses that represent a spectrum of inflammatory and fibrotic diseases with related features. These other disorders include eosinophilia myalgia syndrome, toxic oil syndrome, eosinophilic fasciitis, morphea, and linear scleroderma (reviewed in ref. 1). SSc is now generally divided into two major clinical subsets: limited SSc and diffuse SSc, based on the extent of skin involvement. Diffuse SSc involves the skin proximal to the elbow or knee and frequently involves the trunk, whereas limited SSc involves skin of the hands, forearms, legs below the knees, and face. The course and prognosis are quite variable from patient to patient and are related to disease subset, with diffuse disease having a worse prognosis. The overall mortality of SSc is reported to be 50% at 10 yr (2).
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Wright, T.M. (2000). Systemic Sclerosis. In: Tsokos, G.C. (eds) Principles of Molecular Rheumatology. Current Molecular Medicine, vol 1. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-59259-018-6_23
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DOI: https://doi.org/10.1007/978-1-59259-018-6_23
Publisher Name: Humana Press, Totowa, NJ
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