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Systemic scleroderma
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{{Short description|Accumulation of collagen in the skin and internal organs}} {{Infobox medical condition (new) | name = Systemic scleroderma | synonyms = Diffuse scleroderma, systemic sclerosis, Curzio's Syndrome | image = Riehl Zumbusch Tafel LXIX (1).jpg | caption = Patient with systemic scleroderma | symptoms = | complications = | onset = | duration = | types = | causes = | risks = | diagnosis = | differential = | prevention = | treatment = | medication = | prognosis = | frequency = | deaths = }} '''Systemic [[scleroderma]],''' or '''systemic sclerosis''', is an [[systemic autoimmune disease|autoimmune]] [[Rheumatology|rheumatic disease]] characterised by excessive production and accumulation of [[collagen]], called [[fibrosis]], in the skin and internal organs and by injuries to small [[Artery|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]]. [[Organ (anatomy)|Visceral organs]], including the [[kidney]]s, [[heart]], [[lung]]s, and [[Human gastrointestinal tract|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.<ref>{{cite journal|date=July 2013|title=Cancer incidence in systemic sclerosis: meta-analysis of population-based cohort studies|journal=Arthritis Rheum.|volume=65|issue=7|pages=1913β21|doi=10.1002/art.37969|pmid=23576072|vauthors=Onishi A, Sugiyama D, Kumagai S, Morinobu A|doi-access=free}}</ref> Survival rates have greatly increased with effective treatment for [[kidney failure]]. Therapies include [[immunosuppressive drug]]s, and in some cases, [[glucocorticoid]]s.<ref name="Harrison's">{{cite book|editor1=Longo, Dan L. |editor2=Kasper, Dennis L |editor3=Fauci, Anthony |editor4=Hauser, Stephen L.|title=Harrison's Principles of Internal Medicine |edition=16th |publisher=McGraw-Hill|location=New York|date=July 2011 |orig-date=2005|isbn=978-0-07-174889-6|display-editors=etal}}</ref>
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