• Eleftherios Pelechas
  • Evripidis Kaltsonoudis
  • Paraskevi V. Voulgari
  • Alexandros A. Drosos


Scleroderma or systemic sclerosis (SCL) is an autoimmune disorder of unknown aetiology, characterised by fibrosis and microvascular injury in affected organs. The hallmark of the disease is thickening and tightness of the skin and of subcutaneous tissue. SCL may be confined to the skin (localised) or it may be generalised (systemic sclerosis). In the generalised form, involvement virtually of any organ systems can occur, most importantly the skin, blood vessels, lungs, kidneys, gastrointestinal tract, and the heart. SCL is further subdivided into limited and diffuse (depending on the extent of cutaneous involvement). A subset of patients with limited SCL have prominent calcinosis cutis, Raynaud’s phaenomenon (RP), esophageal dysmotility, sclerodactyly and telangiectasias and is called CREST syndrome. However, these features may also be seen in patients with diffuse SCL.

References and Further Reading

  1. Abou-Raya A, Abou-Raya S, Helmii M. Statins: potentially useful in therapy of systemic sclerosis-related Raynaud’s phenomenon and digital ulcers. J Rheumatol. 2008;35:1801–8.PubMedGoogle Scholar
  2. Airo P, Ceribelli A, Cavazzana I, et al. Malignancies in Italian patients with systemic sclerosis positive for anti-RNA polymerase III antibodies. J Rheumatol. 2011;38:1329–34.CrossRefGoogle Scholar
  3. Akram MR, Handler CE, Williams M, et al. Angiographically proven coronary artery disease in scleroderma. Rheumatology (Oxford). 2006;45: 163:1395–8.CrossRefGoogle Scholar
  4. Alamanos Y, Tsifetaki N, Voulgari PV, Siozos C, Tsamandouraki K, Alexiou GA, Drosos AA. Epidemiology of systemic sclerosis in Northwest Greece 1981 to 2002. Semin Arthritis Rheum. 2005;34(5):714–20.CrossRefGoogle Scholar
  5. Arnett FC, Howard RF, Tan F, et al. Increased prevalence of systemic sclerosis in a Native American tribe in Oklahoma: association with an Amerindian HLA haplotype. Arthritis Rheum. 1996;39:1362–70.CrossRefGoogle Scholar
  6. Avouac J, Guerini H, Wipff J, et al. Radiological hand involvement in systemic sclerosis. Ann Rheum Dis. 2006;65:1088–92.CrossRefGoogle Scholar
  7. Avouac J, Walker U, Tyndall A, et al. Characteristics of joint involvement and relationships with systemic inflammation in systemic sclerosis: results from the EULAR Scleroderma Trial and Research Group (EUSTAR) database. J Rheumatol. 2010;37:1488–501.CrossRefGoogle Scholar
  8. Berezne A, Ranque B, Valeyre D, et al. Therapeutic strategy combining intravenous cyclophosphamide followed by oral azathioprine to treat worsening interstitial lung disease associated with systemic sclerosis: a retrospective multicenter open-label study. J Rheumatol. 2008;35:1064–72.PubMedGoogle Scholar
  9. Botzoris V, Drosos AA. Management of Raynaud’s phenomenon and digital ulcers in systemic sclerosis. Joint Bone Spine. 2011;78(4):341–6.CrossRefGoogle Scholar
  10. Bournia VK, Evangelou K, Sfikakis PP. Therapeutic inhibition of tyrosine kinases in systemic sclerosis: a review of published experience on the first 108 patients treated with imatinib. Semin Arthritis Rheum. 2013;42:377–90.CrossRefGoogle Scholar
  11. Bouros D, Wells AU, Nicholson AG, et al. Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome. Am J Respir Crit Care Med. 2002;165:1581–6.CrossRefGoogle Scholar
  12. Clements PJ, Becvar R, Drosos AA, Ghattas L, Gabrielli A. Assessment of gastrointestinal involvement. Clin Exp Rheumatol. 2003;21(3 Suppl 29):S15–8.PubMedGoogle Scholar
  13. Della Rossa A, Valentini G, Bombardieri S, et al. European multicenter study to define disease activity criteria for systemic sclerosis. I. Clinical and epidemiological features of 290 patients from 19 centres. Ann Rheum Dis. 2001;60(6):585–91.CrossRefGoogle Scholar
  14. Hoogen F, Khanna D, Fransen J, 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.CrossRefGoogle Scholar
  15. LeRoy EC, Black C, Fleischmajer R, et al. Scleroderma (systemic sclerosis): classification, subsets, and pathogenesis. J Rheumatol. 1988;15:202–5.PubMedGoogle Scholar
  16. Steen VD, Medsger TA Jr. Severe organ involvement in systemic sclerosis with diffuse scleroderma. Arthritis Rheum. 2000;43:2437–44.CrossRefGoogle Scholar
  17. Valentini G, Della Rossa A, Bombardieri S, et al. European multicenter study to define disease activity criteria for systemic sclerosis. II. Identification of disease activity variables and development of preliminary activity indexes. Ann Rheum Dis. 2001;60(6):592–8.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Eleftherios Pelechas
    • 1
  • Evripidis Kaltsonoudis
    • 1
  • Paraskevi V. Voulgari
    • 1
  • Alexandros A. Drosos
    • 1
  1. 1.Rheumatology Clinic - Department of Internal MedicineUniversity of IoanninaIoanninaGreece

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