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Epidemiology, Environmental, and Infectious Risk Factors

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Scleroderma

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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. Clinical classification of SSc is divided into two main groups: limited and diffuse cutaneous disease. 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/or 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 along with inborn/genetic and acquired/environmental risk factors.

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Authors and Affiliations

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

    Bochra Jandali & Maureen D. Mayes

  2. Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Universite Paris Descartes, Paris, France

    Luc Mouthon

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  1. Bochra Jandali
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  2. Luc Mouthon
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  3. Maureen D. Mayes
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Correspondence to Maureen D. Mayes .

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Editors and Affiliations

  1. INSERMU1016, Université Paris Cité,, Service de Rhumatologie, Hôpital Cochin,, Paris, France

    Yannick Allanore

  2. Department of Internal Medicine, University of Michigan–Ann Arbor, Ann Arbor, MI, USA

    John Varga

  3. Centre for Rheumatology, University College London, Royal Free Campus, London, UK

    Christopher P. Denton

  4. Department of Allergy and Rheumatology, Nippon Medical School, Tokyo, Tokyo, Japan

    Masataka Kuwana

  5. Rheumatology, Stanford Medicine, Redwood City, CA, USA

    Lorinda Chung

  6. Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA

    Ami A. Shah

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Jandali, B., Mouthon, L., Mayes, M.D. (2024). Epidemiology, Environmental, and Infectious Risk Factors. In: Allanore, Y., Varga, J., Denton, C.P., Kuwana, M., Chung, L., Shah, A.A. (eds) Scleroderma. Springer, Cham. https://doi.org/10.1007/978-3-031-40658-4_2

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