Clinical appearance of acrosclerotic piece-meal necrosis of the thumb in a patient with systemic sclerosis.
Specialty
Rheumatology
Systemic scleroderma, or systemic sclerosis, is an autoimmune rheumatic disease characterised by excessive production and accumulation of collagen, called fibrosis, in the skin and internal organs and by injuries to small arteries. There are two major subgroups of systemic sclerosis based on the extent of skin involvement: limited and diffuse. The limited form affects areas below, but not above, the elbows and knees with or without involvement of the face. The diffuse form also affects the skin above the elbows and knees and can also spread to the torso. Visceral organs, including the kidneys, heart, lungs, and gastrointestinal tract can also be affected by the fibrotic process.
Prognosis is determined by the form of the disease and the extent of visceral involvement. Patients with limited systemic sclerosis have a better prognosis than those with the diffuse form. Death is most often caused by lung, heart, and kidney involvement. The risk of cancer is increased slightly.[1]
Survival rates have greatly increased with effective treatment for kidney failure. Therapies include immunosuppressive drugs, and in some cases, glucocorticoids.[2]
^Onishi A, Sugiyama D, Kumagai S, Morinobu A (July 2013). "Cancer incidence in systemic sclerosis: meta-analysis of population-based cohort studies". Arthritis Rheum. 65 (7): 1913–21. doi:10.1002/art.37969. PMID 23576072.
^Longo, Dan L.; Kasper, Dennis L; Fauci, Anthony; Hauser, Stephen L.; et al., eds. (July 2011) [2005]. Harrison's Principles of Internal Medicine (16th ed.). New York: McGraw-Hill. ISBN 978-0-07-174889-6.
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