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Juvenile Systemic Sclerosis

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Scleroderma

Abstract

Juvenile systemic sclerosis (jSSc), is a rare multisystem connective tissue disease. Approximately 5–10% of all adults with systemic sclerosis (SSc) report the onset of the disease during childhood. It has a variety of clinical manifestations, sometimes different from the adult form. Mixed connective tissue disease (MCTD) and overlap syndromes have features of jSSc and sometimes even fulfil the criteria of jSSc (Zulian F, Woo P, Athreya BH, et al. The Pediatric Rheumatology European Society/American College of Rheumatology/European League against Rheumatism provisional classification criteria for juvenile systemic sclerosis. Arthritis Rheum. 2007;57(2):203–12). It is important to differentiate from juvenile localised scleroderma, which is much more common in the paediatric population, it occurs in 4.7–20 per 100,000 and it is 10 times more frequent the jSSc. It has in most cases a more benign course with the exception of pansclerotic morphea (Zulian F, Athreya BA, Laxer R, et al. Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study. Rheumatology. 2006;45:614–20).

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Authors and Affiliations

  1. Am Schön Klinik Hamburg. Hamburger Zentrum Für Kinder – und Jugendrheumatologie, Hamburg, Germany

    Ivan Foeldvari MD

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  1. Ivan Foeldvari MD
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Correspondence to Ivan Foeldvari MD .

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Editors and Affiliations

  1. Feinberg School of Medicine, Northwestern University, E. Huron Street 240, Chicago, 60611-2909, Illinois, USA

    John Varga

  2. Royal Free and University College, Medical School, University College London, Rowland Street, London, NW3 2PF, United Kingdom

    Christopher P. Denton

  3. School of Medicine, Div. Rheumatology, Johns Hopkins University, Eastern Avenue 5200, Baltimore, 21224, Maryland, USA

    Fredrick M. Wigley

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Foeldvari, I. (2012). Juvenile Systemic Sclerosis. In: Varga, J., Denton, C., Wigley, F. (eds) Scleroderma. Springer, Boston, MA. https://doi.org/10.1007/978-1-4419-5774-0_10

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  • DOI: https://doi.org/10.1007/978-1-4419-5774-0_10

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  • Online ISBN: 978-1-4419-5774-0

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