Systemisk sklerose (sklerodermi)
Systemisk sklerose er en bindevevssykdom som angriper mange organer og gir uttalt bindevevsdannelse, såkalt sklerose. Huden angripes nesten alltid. Prognosen avhenger av hvilken type systemisk sklerose det er.
Sist oppdatert:
13. juli 2020
Hva er systemisk sklerose?
Tilstanden kalles også sklerodermi og er en systemisk bindevevssykdom. Systemisk vil si at det er en sykdom som angriper mange organer. Sykdommen fører til bindevevsdannelse i hud, blodårer og indre organer. Over tid medfører den økte bindevevsdannelsen at huden blir stiv og ubevegelig, og det utvikles funksjonssvikt i indre organer.
Det er en sjelden sykdom, med tydelige geografiske variasjoner i forekomst. I USA er det beregnet at det finnes cirka 275 personer med denne diagnosen per en million voksne. Tilsvarende tall i Frankrike er 150, og i England 88 tilfeller per en million voksne - forekomsten er med andre ord mer enn tre ganger høyere i USA sammenlignet med England.
Sykdommen opptrer oftest blant kvinner i aldersgruppen 30-40 år og hos noe eldre menn. Man regner at 85 prosent oppstår i aldersgruppen fra 20-60 år, men tilstanden forekommer både hos barn og eldre.
Vil du vite mer
Dette dokumentet er basert på det profesjonelle dokumentet Systemisk sklerose . Referanselisten for dette dokumentet vises nedenfor
- Kowal-Bielecka O, Fransen J, Avouac J EUSTAR Coauthors, et alUpdate of EULAR recommendations for the treatment of systemic sclerosisAnnals of the Rheumatic Diseases 2017;76:1327-1339. ard.bmj.com
- Schwartz RA. Systemic sclerosis. Medscape, last updated Mar 13, 2018. emedicine.medscape.com
- Varga J. Overview of the clinical manifestations of systemic sclerosis (scleroderma) in adults. UpToDate, last updated Jan 09, 2015. UpToDate
- Chifflot H, Fautrel B, Sordet C, et al. Incidence and prevalence of systemic sclerosis: a systematic literature review. Semin Arthritis Rheum 2008; 37: 223-35. pmid:17692364 PubMed
- Gran JT, Molberg Ø, Midtvedt Ø. Patofysiologiske aspekter ved systemisk sklerose. Tidsskr Nor Lægeforen 2005; 125: 2622-3. PubMed
- Kuwana M, Okazaki Y, Yasuoka H, Kawakami Y, Ikeda Y. Defective vasculogenesis in systemic sclerosis. Lancet 2004; 364: 603-10. PubMed
- Varga J. Overview of the clinical manifestations of systemic sclerosis (scleroderma) in adults. UpToDate, last updated Jan 06, 2017. UpToDate
- Varga J. Diagnosis and differential diagnosis of systemic sclerosis (scleroderma) in adults. UpToDate, last updated Jan 05, 2017. UpToDate
- Gran JT, Midtvedt Ø. Iskemiske sår ved systemisk bindevevssykdom. Tidsskr Nor Lægeforen 2006; 126: 3089-92. PubMed
- Kuwana M. Circulating Anti-Nuclear Antibodies in Systemic Sclerosis: Utility in Diagnosis and Disease Subsetting. J Nippon Med Sch 2017; 84(2): 56-63. pmid:28502960 PubMed
- Muangchan C, Harding S, Khimdas S, Bonner A, Baron M, Pope J. C - reactive protein (CRP) is associated with high disease activity in systemic sclerosis: results from the Canadian Scleroderma Research Group (CSRG). Arthritis Care Res (Hoboken). 2012; 64(9):1405-14. PubMed
- Derrett-Smith EC, Nihtyanova SI, Harvey J, et al. Revisiting ANCA-associated vasculitis in systemic sclerosis: clinical, serological and immunogenetic factors. Rheumatology (Oxford) 2013; 52:1824. PubMed
- Denton CP. Overview of the treatment and prognosis of systemic sclerosis (scleroderma) in adults. UpToDate, last updated Oct 10, 2014. UpToDate
- Charles C, Clements P, Furst DE. Systemic sclerosis: hypothesis-driven treatment strategies. Lancet 2006; 367: 1683-91. PubMed
- García de la Peña Lefebvre P, Nishishinya MB, Pereda CA, Loza E, Sifuentes Giraldo WA, Román Ivorra JA, et al. Efficacy of Raynaud's phenomenon and digital ulcer pharmacological treatment in systemic sclerosis patients: a systematic literature review. Rheumatol Int. 2015; 35(9):1447-59. PubMed
- Tashkin DP, Elashoff R, Clements PJ, et al. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med 2006; 354: 2655-66. New England Journal of Medicine
- Martinez FJ, McCune J. Cyclophosphamide for scleroderma lung disease. N Engl J Med 2006; 354: 2707-9. PubMed
- Jordan S, Distler JHW, Maurer B, et al. Effects and safety of rituximab in systemic sclerosis: An analysis from the European Scleroderma Trial and Research (EUSTAR) group. Ann Rheum Dis 2015 Jun; 74:1188. Doi: 10.1136/annrheumdis-2013-204522 DOI
- Tashkin DP, Roth MD, Clements PJ et al. Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomised controlled, double-blind, parallel group trial. Lancet respir Med 2016; 4: 708. doi:27469583
- Distler O, Highland KB, Gahlemann M, et al. Nintedanib for Systemic Sclerosis-Associated Interstitial Lung Disease. N Engl J Med. 2019 PMID: 31112379 PubMed
- van Laar JM, Farge D, Sont JK, et al. Autologous hematopoietic stem cell transplantation vs intravenous pulse cyclophosphamide in diffuse cutaneous systemic sclerosis: a randomized clinical trial. JAMA. 2014 Jun 25;311(24):2490-8. PubMed
- Sullivan KM., Goldmuntz EA., Keyes-Elstein L. et al.. Myeloablative Autologous Stem-Cell Transplantation for Severe Scleroderma. N Engl J Med 2018; 378: 35-47. pmid:29298160 PubMed
- Onishi A, Sugiyama D, Kumagai S, et al. Cancer incidence in systemic sclerosis: meta-analysis of population-based cohort studies. Arthritis Rheum 2013 Jul;65(7):1913-21. doi: 10.1002/art.37969. DOI
- Tennøe AH, Murbræch K, Andreassen JC, et al. Left ventricular diastolic dysfunction predicts mortality in patients with systemic sclerosis. Journal of the American College of Cardiology, volume 72, issue 15, October 2018.
- Tyndall AJ, Bannert B, Vonk M, et al. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis 2010; 69:1809. PubMed