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Systemic Sclerosis

  • Matteo ZanardelliEmail author
  • Zoulikha Zarrab
  • Aldona Pietrzak

Abstract

Systemic sclerosis is a chronic multisystemic progressive disease that affects the skin and internal organs. Generally, Raynaud’s phenomenon and vascular changes are the first clinical manifestations followed by sclerotic and fibrotic changes. It is a rare disease: the incidence is 8–12 patients per million population and about 75 % of patients are woman. It has the highest case-specific mortality of any of the autoimmune rheumatic diseases: the 5-year survival is about 50–70 % when pulmonary hypertension is present. It is determined by the involvement of the heart and lung (pulmonary fibrosis, pneumonia) and in some populations of higher frequency of the kidney (uraemia, malignant hypertension).

Further Reading

  1. Beyer C, Distler O, Distler JH. Innovative antifibrotic therapies in systemic sclerosis. Curr Opin Rheumatol. 2012;24(3):274–80.CrossRefGoogle Scholar
  2. Denton CP, Black CM. Scleroderma (systemic sclerosis) In: Wolff K, Goldsmith LA, Katz SI, Gilchrist BA, Paller AS, Leffell DJ, editors. Fitzpatrick’s Dermatology in General Medicine. 7th ed. New York, NY: McGraw-Hill Medical Publishing Division; 2008. pp. 1553–62.Google Scholar
  3. Fett N. Scleroderma: nomenclature, etiology, pathogenesis, prognosis, and treatments: facts and controversies. Clin Dermatol. 2013;31(4):432–7.CrossRefGoogle Scholar
  4. Fox P, Chung L, Chang J. Management of the hand in systemic sclerosis. J Hand Surg [Am]. 2013;38(5):1012–6.CrossRefGoogle Scholar
  5. Frech TM, Shanmugam VK, Shah AA, Assassi S, Gordon JK, Hant FN, Hinchcliff ME, Steen V, Khanna D, Kayser C, Domsic RT. Treatment of early diffuse systemic sclerosis skin disease. Clin Exp Rheumatol. 2013;31(2 Suppl 76):166–71.PubMedPubMedCentralGoogle Scholar
  6. Haustein UF. Systemic sclerosis: scleroderma. In: Katsambas A, Lotti TM, editors. European handbook of dermatological treatments. 2nd ed. Berlin/Heidelberg: Springer; 2003.Google Scholar
  7. Luo Y, Wang Y, Wang Q, Xiao R, Lu Q. Systemic sclerosis: genetics and epigenetics. J Autoimmun. 2013;41:161–7.CrossRefGoogle Scholar
  8. Poole JL, Macintyre NJ, Deboer HN. Evidence-based management of hand and mouth disability in a woman living with diffuse systemic sclerosis (scleroderma). Physiother Can. 2013;65(4):317–20.CrossRefGoogle Scholar
  9. Ramos-Casals M, Fonollosa-Pla V, Brito-Zerón P, Sisó-Almirall A. Targeted therapy for systemic sclerosis: how close are we? Nat Rev Rheumatol. 2010;6(5):269–78.CrossRefGoogle Scholar
  10. Rongioletti F, Rampini P, Parodi A, Rebora A. Papular mucinosis associated with generalized morphoea. Br J Dermatol. 1999;141(5):905–8.CrossRefGoogle Scholar
  11. van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, Matucci-Cerinic M, Naden RP, Medsger Jr TA, Carreira PE, Riemekasten G, Clements PJ, Denton CP, Distler O, Allanore Y, Furst DE, Gabrielli A, Mayes MD, van Laar JM, Seibold JR, Czirjak L, Steen VD, Inanc M, Kowal-Bielecka O, Müller-Ladner U, Valentini G, Veale DJ, Vonk MC, Walker UA, Chung L, Collier DH, Csuka ME, Fessler BJ, Guiducci S, Herrick A, Hsu VM, Jimenez S, Kahaleh B, Merkel PA, Sierakowski S, Silver RM, Simms RW, Varga J, Pope JE. 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative. Arthritis Rheum. 2013a;65(11):2737–47.CrossRefGoogle Scholar
  12. van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, Matucci-Cerinic M, Naden RP, Medsger Jr TA, Carreira PE, Riemekasten G, Clements PJ, Denton CP, Distler O, Allanore Y, Furst DE, Gabrielli A, Mayes MD, van Laar JM, Seibold JR, Czirjak L, Steen VD, Inanc M, Kowal-Bielecka O, Müller-Ladner U, Valentini G, Veale DJ, Vonk MC, Walker UA, Chung L, Collier DH, Ellen Csuka M, Fessler BJ, Guiducci S, Herrick A, Hsu VM, Jimenez S, Kahaleh B, Merkel PA, Sierakowski S, Silver RM, Simms RW, Varga J, Pope JE. 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative. Ann Rheum Dis. 2013b;72(11):1747–55.CrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  • Matteo Zanardelli
    • 1
    Email author
  • Zoulikha Zarrab
    • 2
    • 3
  • Aldona Pietrzak
    • 4
  1. 1.Department of Neuroscience and Psychology, Drug Research and Child HealthUniversità di FirenzeFirenzeItaly
  2. 2.University of Rome “G. Marconi”RomeItaly
  3. 3.Centro Studi per la Ricerca Multidisciplinare Rigenerativa (CSRMR)RomeItaly
  4. 4.Department of Dermatology, Venereology and Paediatric DermatologyMedical University of LublinLublinPoland

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