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Epidemiology and Environmental Risk Factors

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Book cover Scleroderma

Abstract

Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterized by excessive collagen deposition in the skin and internal organs with associated vasculopathy and autoantibody production (Bolster MB, Silver RS. Clinical features of systemic sclerosis. In: Hochberg MC, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH, editors. Rheumatology. 5th ed. Philadelphia: Mosby: Elsevier; 2011. p. 1373–86). Classification of SSc is divided into two main groups: limited and diffuse cutaneous disease (LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger Jr TA, et al. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol. 1988;15(2):202–5). The limited form is characterized by skin thickening that is confined to areas distal to the elbows and knees and generally is associated with less severe internal organ involvement. The diffuse form involves skin thickening proximal to the elbows and the knees as well as distal areas and is associated with more severe organ damage. This chapter will focus on the epidemiology of systemic sclerosis including both limited and diffuse cutaneous forms.

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Authors and Affiliations

  1. Internal Medicine, Division of Rheumatology and Immunogenetics, University of Texas Health Science Center – Houston, Houston, TX, USA

    Jammie K. Barnes MD & Maureen D. Mayes MD, MPH

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  1. Jammie K. Barnes MD
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  2. Maureen D. Mayes MD, MPH
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Correspondence to Jammie K. Barnes 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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Barnes, J.K., Mayes, M.D. (2012). Epidemiology and Environmental Risk Factors. In: Varga, J., Denton, C., Wigley, F. (eds) Scleroderma. Springer, Boston, MA. https://doi.org/10.1007/978-1-4419-5774-0_4

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