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Evolving Concepts of Diagnosis and Classification

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Scleroderma

Abstract

Systemic sclerosis (SSc) is a rare connective tissue disease characterized by fibrosis of the skin and internal organs and a vasculopathy affecting the micro- and macro-vasculature. The pathogenesis of SSc involves the interplay between vascular injury and dysregulation of the immune response with resultant fibrosis of various target organs. More than 90% of patients with SSc suffer from Raynaud’s phenomenon, a reversible vasospastic disorder induced by cold or stress, which results in typical white, blue, and red color changes of the distal extremities from decreased perfusion (Block JA, Sequeira W, 2001. Raynaud’s phenomenon. Lancet 357(9273), 2042–8). Other clinical features that are common in patients with SSc in addition to cutaneous sclerosis include pulmonary disease (interstitial lung disease and pulmonary hypertension), gastrointestinal dysmotility and malabsorption, digital ulcerations, inflammatory myositis and arthritis, cardiac and renal disease.

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Authors and Affiliations

  1. Stanford University School of Medicine, Palo Alto, CA, USA

    Lorinda Chung MD, MS

  2. Department of Rheumatology, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands

    Jaap Fransen MD

  3. Sint Maartenskliniek, Nijmegen, The Netherlands

    Frank H. J. Van den Hoogen MD, PhD

Authors
  1. Lorinda Chung MD, MS
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  2. Jaap Fransen MD
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  3. Frank H. J. Van den Hoogen MD, PhD
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Corresponding author

Correspondence to Lorinda Chung MD, MS .

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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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Chung, L., Fransen, J., Van den Hoogen, F.H.J. (2012). Evolving Concepts of Diagnosis and Classification. In: Varga, J., Denton, C., Wigley, F. (eds) Scleroderma. Springer, Boston, MA. https://doi.org/10.1007/978-1-4419-5774-0_7

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  • DOI: https://doi.org/10.1007/978-1-4419-5774-0_7

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