Systemic Sclerosis (SSc)

  • Nages Nagaratnam
  • Kujan Nagaratnam
  • Gary Cheuk
Reference work entry

Abstract

Epidemiological studies have underscored the incidence of SSc in the sixth, seventh and eighth decades of life. The pathogenesis of systemic sclerosis (SSc) is characterised by immune and endothelial activation, vascular dysfunction and overproduction of extracellular matrix. The late onset is clinically and immunologically different from the early onset. There are two subsets of SSc, diffuse cutaneous SSc (dcSSc) which is associated with progressive fibrosis of skin and internal organs and the other limited cutaneous.

Keywords

Systemic sclerosis Late onset Early onset Diffuse cutaneous Diffuse cutaneous CREST syndrome 

References

  1. 1.
    Dalziel JA, Wilcock GH. Evidence to suggest the incidence of progressive systemic sclerosis in the elderly is more common than with the younger age group. Postgrad Med J. 1979;55(84):192–195.CrossRefPubMedPubMedCentralGoogle Scholar
  2. 2.
  3. 3.
    Mayes MD, Lacey JU, Beebee-Dimmer J, Gillepsie BW, Cooper B, Liang TJ, et al. Prevalence incidence survival and disease characteristics of systemic sclerosis in a large US population. Arthritis Rheum. 2003;48:246–55.Google Scholar
  4. 4.
    Laing TJ, Gillepsie BW, Toth MB, Mayes MD, Gallavan RH Jr, Burns CJ, et al. Racial differences in scleroderma among women in Michigan. Arthritis Rheum. 1997;40(4):734–42.CrossRefPubMedGoogle Scholar
  5. 5.
    Medsger TA, Jr, Masi AT. Epidemiology of systemic sclerosis (scleroderma). Ann Intern Med. 1971;74(5):714–21.CrossRefPubMedGoogle Scholar
  6. 6.
    Denton CP. Pathogenesis of systemic sclerosis (scleroderma). UpToDate. http://www.update.com/contents/pathogensis-of-systemic-sclerosis-scleroderma. Accessed 27April 2015.
  7. 7.
    Yamamoto T. Scleroderma-pathophysiology. Eur J Dermatol. 2009;19(1):14–24 .PubMedGoogle Scholar
  8. 8.
    Manno RL, Wigley FM, Hummers LK. Late-onset scleroderma. Arthritis Rheum. 2009;60 Suppl 10:1733  https://doi.org/10.1002/art.26807.Google Scholar
  9. 9.
    Manno RL, Wigley FM, Gelber AC, Hummers LK. Late-onset systemic sclerosis. J Rheumatol. 2011;38(7):1317–25.CrossRefPubMedPubMedCentralGoogle Scholar
  10. 10.
    Hugle T, Schuetz P, Daikeler T, Tyndall A, Matucci-Cerinic M, Walker UA, et al. Late-onset systemic sclerosis – a systematic survey of the EULAR scleroderma trials and research group database. Rheumatology. (2010).  https://doi.org/10.1093/rheumatology/keq321.
  11. 11.
    Hachulla E, Launay D. Diagnosis and classification of systemic sclerosis. Clin Rev Allergy Immunol. 2011; 40(2):78–83.CrossRefPubMedGoogle Scholar
  12. 12.
    Systemic sclerosis in old age. Br Med J. 1979;2(6201):1313–4.Google Scholar
  13. 13.
    Bryan C, Knight C, Black CM, Silman AI. Prediction of five-year survival following presentation with scleroderma: development of a simple model using three disease factors at first visit. Arthritis Rheum. 1999; 42:2660–5.CrossRefPubMedGoogle Scholar
  14. 14.
    Mayes MD, Lacey JV Jr, Beebe-Dimmer J, Gillepsie BW, Cooper B, Laing TJ, et al. Prevalence, incidence, survival and disease characteristics of systemic sclerosis in a large US population. Arthritis Rheum. 2003;48(8):2246–55CrossRefPubMedGoogle Scholar
  15. 15.
    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(6):831–4.CrossRefPubMedGoogle Scholar
  16. 16.
    Czirjak L, Nagy Z, Szegedi G. Systemic sclerosis in the elderly. Clin Rheumatol. 1992;11(4):483–5.CrossRefPubMedGoogle Scholar
  17. 17.
    Alba MA, Valasco C, Simeon CP, Folollosa V, Trapiella L, Egurbide MV, et al. Early-versus late-onset systemic sclrerosis: differences in clinical presentation and outcome in 1037 patients. Medivoine (Baltimore). 2014;93(2):73–81.CrossRefGoogle Scholar
  18. 18.
    University of Maryland Medical Center. Scleroderma. http://umm.edu/health/medical/reports/articles/scleroderma accessed on 25 September, 2015.
  19. 19.
    Hummers LK, Wriley F. Scleroderma treatment options. http://www.hopkinsscleroderma.org/patients/scleroderma-treatment-options/ accessed on 25 September 2015.
  20. 20.
    Hummers LK, Wigley F. Management of Raynaud’s phenomenon and digital ischaemic lesions in scleroderma. Rheum Dis Clin North Am. 2003;29:293–313.CrossRefPubMedGoogle Scholar
  21. 21.
    Korn JH. Scleroderma. A treatable disease. Cleve Clin J Med. 2003;70:954.,956, 958.CrossRefPubMedGoogle Scholar
  22. 22.
    Korn JH, Mayes M, Matucci CM, Cerenic M, Rainisio M, Pope J, et al. Digital ulcers in systemic sclerosis: Prevention by treatment with bosetan, an oral endothelin receptor antagonist. Arthritis Rheum. 2004;50:3985–3993.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Nages Nagaratnam
    • 1
  • Kujan Nagaratnam
    • 1
  • Gary Cheuk
    • 2
  1. 1.The University of SydneyWestmead Clinical SchoolWestmeadAustralia
  2. 2.Rehabilitation and Aged Care ServiceBlacktown-Mt Druitt HospitalMount DruittAustralia

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