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Critical Appraisal of the Utility and Limitations of Animal Models of Scleroderma

  • Scleroderma (J Varga, Section Editor)
  • Published:
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Abstract

Scleroderma, also called systemic sclerosis (SSc), is an autoimmune connective tissue disease characterized by abnormality of the immune system, cardiovascular disorder, and fibrosis of the skin, lung, kidney, and other internal organs. Skin symptom and stiffness of the skin with accumulation of collagen cause severe disability, and cardiovascular and lung complications lead to high mortality. Despite recent progress, therapies for these complications in SSc are limited and the underlying mechanisms are poorly understood. Animal models are one strategy to identify molecular mechanisms and pathways underlying these complications of SSc and could provide a mechanistic basis for a design of clinical trials. However, although several animal models have been reported to reproduce some aspects of SSc, none of them recapitulates all of the pathological features of SSc. In this review, we describe previously reported animal models of scleroderma and discuss their utility and limitations in efforts to understand the mechanisms underlying scleroderma and develop new treatments for this disease.

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  1. Department of Medicine, University of California, San Francisco, CA, 94143, USA

    Kazuyuki Tsujino & Dean Sheppard

  2. Department of Respiratory Medicine, Allergy and Rheumatic Diseases, Osaka University Graduate School of Medicine, Suita, Osaka, Japan

    Kazuyuki Tsujino

  3. Department of Medicine, Lung Biology Center, University of California, San Francisco, Box 2922, San Francisco, CA, 94143-2922, USA

    Kazuyuki Tsujino & Dean Sheppard

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Tsujino, K., Sheppard, D. Critical Appraisal of the Utility and Limitations of Animal Models of Scleroderma. Curr Rheumatol Rep 18, 4 (2016). https://doi.org/10.1007/s11926-015-0553-9

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