Abstract
Systemic sclerosis (SSc) is a rare systemic chronic disease characterized by widespread fibrosis of skin and internal organs, microvasculopathy, and autoantibodies that frequently affects internal organs and quality of life and survival. Disease-related autoantibodies, anti-topoisomerase I, anticentromere, and anti-RNA polymerase III antibodies, Raynaud’s phenomenon, and microvasculopathy, as detected by nailfold capillaroscopy, appear before skin fibrosis and help in early diagnosis. Based on the extent of skin involvement, SSc is divided into limited cutaneous SSc and diffuse cutaneous SSc. SSc-related autoantibodies are associated with manifestations and help in the management of SSc patients. In this chapter we review epidemiology, pathogenesis, clinical manifestations, specific organ involvement and assessment tools, diagnosis, differential diagnosis, and treatment.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Royle JG, et al. The incidence, prevalence, and survival of systemic sclerosis in the UK Clinical Practice Research Datalink. Clin Rheumatol. 2018;37(8):2103–11.
Meier FM, et al. Update on the profile of the EUSTAR cohort: an analysis of the EULAR Scleroderma Trials and Research group database. Ann Rheum Dis. 2012;71(8):1355–60.
Morgan ND, et al. Clinical and serological features of systemic sclerosis in a multicenter African American cohort: analysis of the genome research in African American scleroderma patients clinical database. Medicine (Baltimore). 2017;96(51):e8980.
Marangoni RG, et al. Myofibroblasts in murine cutaneous fibrosis originate from adiponectin-positive intradermal progenitors. Arthritis Rheumatol. 2015;67(4):1062–73.
Sakkas LI, Chikanza IC, Platsoucas CD. Mechanisms of disease: the role of immune cells in the pathogenesis of systemic sclerosis. Nat Clin Pract Rheumatol. 2006;2(12):679–85.
Sakkas LI, Bogdanos DP. Systemic sclerosis: new evidence re-enforces the role of B cells. Autoimmun Rev. 2016;15(2):155–61.
Bhattacharyya S, et al. Tenascin-C drives persistence of organ fibrosis. Nat Commun. 2016;7:11703.
Dore A, et al. Significance of palpable tendon friction rubs in early diffuse cutaneous systemic sclerosis. Arthritis Care Res (Hoboken). 2013;65(8):1385–9.
Denton CP, Khanna D. Systemic sclerosis. Lancet. 2017;390(10103):1685–99.
Bussone G, Mouthon L. Interstitial lung disease in systemic sclerosis. Autoimmun Rev. 2011;10(5):248–55.
Goh NS, et al. Interstitial lung disease in systemic sclerosis: a simple staging system. Am J Respir Crit Care Med. 2008;177(11):1248–54.
Coghlan JG, et al. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Ann Rheum Dis. 2014;73(7):1340–9.
Galie N, et al. 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: the joint task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J. 2016;37(1):67–119.
Kylhammar D, et al. A comprehensive risk stratification at early follow-up determines prognosis in pulmonary arterial hypertension. Eur Heart J. 2017;39(47):4175–81.
Chung L, et al. Survival and predictors of mortality in systemic sclerosis-associated pulmonary arterial hypertension: outcomes from the pulmonary hypertension assessment and recognition of outcomes in scleroderma registry. Arthritis Care Res (Hoboken). 2014;66(3):489–95.
Sakkas LI, et al. Intestinal involvement in systemic sclerosis: a clinical review. Dig Dis Sci. 2018;63(4):834–44.
Guillevin L, et al. Scleroderma renal crisis: a retrospective multicentre study on 91 patients and 427 controls. Rheumatology (Oxford). 2012;51(3):460–7.
van den Hoogen F, et al. 2013 Classification criteria for systemic sclerosis: an American College of Rheumatology/European league against rheumatism collaborative initiative. Ann Rheum Dis. 2013;72(11):1747–55.
Lazzaroni MG, et al. Malignancies in patients with anti-RNA polymerase III antibodies and systemic sclerosis: analysis of the EULAR scleroderma trials and research cohort and possible recommendations for screening. J Rheumatol. 2017;44(5):639–47.
Valentini G, et al. The European Scleroderma Trials and Research group (EUSTAR) task force for the development of revised activity criteria for systemic sclerosis: derivation and validation of a preliminarily revised EUSTAR activity index. Ann Rheum Dis. 2017;76(1):270–6.
Tyndall A, Fistarol S. The differential diagnosis of systemic sclerosis. Curr Opin Rheumatol. 2013;25(6):692–9.
Wigley F, et al. Case records of the Massachusetts General Hospital. Case 8-2015. A 68-year-old man with multiple myeloma, skin tightness, arthralgias, and edema. N Engl J Med. 2015;372(11):1056–67.
Hao Y, et al. Early mortality in a multinational systemic sclerosis inception cohort. Arthritis Rheumatol. 2017;69(5):1067–77.
Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972–2002. Ann Rheum Dis. 2007;66(7):940–4.
Shah AA, et al. Examination of autoantibody status and clinical features associated with cancer risk and cancer-associated scleroderma. Arthritis Rheumatol. 2015;67(4):1053–61.
Kowal-Bielecka O, et al. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis. 2017;76(8):1327–39.
Fernandez-Codina A, Walker KM, Pope JE. Treatment algorithms for systemic sclerosis according to experts. Arthritis Rheumatol. 2018;70(11):1820–8.
Herrick AL, et al. Treatment outcome in early diffuse cutaneous systemic sclerosis: the European Scleroderma Observational Study (ESOS). Ann Rheum Dis. 2017;76(7):1207–18.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2020 Springer Nature Switzerland AG
About this chapter
Cite this chapter
Sakkas, L.I. (2020). Systemic Sclerosis (Scleroderma). In: Efthimiou, P. (eds) Absolute Rheumatology Review. Springer, Cham. https://doi.org/10.1007/978-3-030-23022-7_11
Download citation
DOI: https://doi.org/10.1007/978-3-030-23022-7_11
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-030-23021-0
Online ISBN: 978-3-030-23022-7
eBook Packages: MedicineMedicine (R0)