Abstract
Systemic scleroderma (SSc, progressive systemic sclerosis) is a generalized connective tissue disease which clinically manifests itself by thickening – fibrosis – and cutaneous sclerosis of various extents (scleroderma) and a typical involvement of multiple internal organs. At the same time, there occur fibrotic and sclerotic changes in vascular walls, disturbances of microvascularization, and disorders of humoral and cellular immunity. It affects 3–8 times more women than men. It develops usually in middle age, with an annual incidence of about 3 − 19 new cases per million population. One of the major clinical manifestations is Raynaud’s phenomenon with trophic changes, skin induration, and involvement of gastrointestinal tract, lungs, heart, and kidneys [2].
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References
Medsger TA. Comment on scleroderma criteria cooperative study. In: Black CM, Myers AR, editors. Systemic sclerosis (Scleroderma). New York: Glower Med; 1985. p. 235–49.
LeRoy EC, Black C, Fleischmajer R, et al. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol. 1988;15:202–5.
Volkov AV, Starovoitova MN, Guseva NG. Characteristics of clinical symptoms and course of systemic scleroderma depending on sex and age of onset. Ter Arkh. 2004;76:7–11.
Hugle T, Schuetz P, Daikeler T, et al. Late-onset systemic sclerosis a systematic survey of the EULAR scleroderma trials and research group database. Rheumatology (Oxford). 2011;50(1):161–5. /Epub 2010 Sep 30./.
Manno RL, Wigley FM, Gelber AC, Hummers LK. Late-age onset systemic sclerosis. J Rheumatol. 2011;38(7):1317–25. /Epub 2011 Jun 17./.
Schachna L, Wigley FM, Chang B, et al. Age and risk of pulmonary arterial hypertension in scleroderma. Chest. 2003;124:2098–104.
Hachulla E, Launay D, Mouthon L, et al. Is pulmonary arterial hypertension really a late complication of systemic sclerosis? Chest. 2009;136(5):1211–9. /Epub 2009 May 8./.
Weinblat ES, Hildebrandt S, Senecal JL. Prognostic significance of anticentromere antibodies and antitopoisomerase I antibodies in Raynaud’s disease. Arthritis Rheum. 1991;34:68–75.
Derk CT, Artlett CM, Jimenez SA. Morbidity and mortality of patients diagnosed with systemic sclerosis after the age of 75: a nested case-control study. Clin Rheumatol. 2006;25:14. /Epub ahead of print./.
Zandman-Goddard G, Tweezer-Zaks N, Shoenfeld Y. New therapeutic strategies for systemic sclerosis a critical analysis of the literature. Clin Dev Immunol. 2005;12(3):165–73.
Dedication
This monography was supported by project of Ministry of Health, concept development of Research Organisation Nr.023728 (Institute of Rheumatology).
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Bečvář, R. (2017). Systemic Scleroderma. In: Rovenský, J. (eds) Gerontorheumatology. Springer, Cham. https://doi.org/10.1007/978-3-319-31169-2_6
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DOI: https://doi.org/10.1007/978-3-319-31169-2_6
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