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Animal models for scleroderma: an update.

Yan Zhang1, Anita C Gilliam

  • 1Department of Dermatology, Case Western Reserve University/ University Hospitals of Cleveland, 10900 Euclid Avenue, Cleveland, OH 44106-5028, USA. acg@po.cwru.edu

Current Rheumatology Reports
|March 14, 2002
PubMed
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Animal models are crucial for understanding scleroderma, a fibrosing disease. This review details various models, including chemically induced, genetically modified, and spontaneous mutations, to study scleroderma pathogenesis and treatments.

Area of Science:

  • * Rheumatology and immunology
  • * Fibrotic disease research

Background:

  • * Scleroderma is a progressive, debilitating fibrosing disease affecting multiple organs.
  • * The exact causes (pathogenesis) of scleroderma are not fully understood.
  • * Animal models are essential tools for investigating scleroderma's mechanisms and evaluating potential therapies.

Purpose of the Study:

  • * To review and categorize various animal models of scleroderma.
  • * To highlight models that mimic key features of human scleroderma, such as fibrosis, inflammation, vascular injury, and immune system changes.
  • * To discuss interventions tested within these models.

Main Methods:

  • * Categorization of animal models based on fibrosis induction methods.
  • * Review of models involving external agents (e.g., vinyl chloride, bleomycin).

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  • * Discussion of genetic models (mutant strain combinations, spontaneous mutations) and transplantation models (graft-versus-host disease).

    • Main Results:

    • * Several distinct categories of animal models effectively recapitulate aspects of scleroderma.
    • * Models include those with chemically induced fibrosis, specific genetic mutations (e.g., integrin alpha 1 null mouse), and immune cell transplantation.
    • * Spontaneous mutation models like the UCD 200 chicken and tight skin mouse also exhibit fibrotic characteristics.

    Conclusions:

    • * A diverse range of animal models exists for studying scleroderma.
    • * Each model offers unique insights into specific pathological features of the disease.
    • * These models are vital for advancing the understanding and treatment of scleroderma.