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Current Rheumatology Reports

, 15:304 | Cite as

Involvement of PDGF in Fibrosis and Scleroderma: Recent Insights from Animal Models and Potential Therapeutic Opportunities

  • Tomoaki Iwayama
  • Lorin E. Olson
SCLERODERMA (J VARGA, SECTION EDITOR)
Part of the following topical collections:
  1. Topical Collection on Scleroderma

Abstract

Fibrosis is the principal characteristic of the autoimmune disease known as scleroderma or systemic sclerosis (SSc). Studies published within the last three years suggest central involvement of platelet-derived growth factors (PDGFs) in SSc-associated fibrosis. PDGFs may also be involved in SSc-associated autoimmunity and vasculopathy. The PDGF signaling pathway is well understood and PDGF receptors are expressed on collagen-secreting fibroblasts and on mesenchymal stem and/or progenitor cells that may affect SSc in profound and unexpected ways. Although much work remains before we fully understand how PDGFs are involved in SSc, there is much interest in using PDGF inhibitors as a therapeutic approach to SSc.

Keywords

PDGF Scleroderma Fibrosis Mouse model Animal models Pericyte Imatinib Neutralizing antibody Therapeutic 

Notes

Disclosure

No potential conflicts of interest relevant to this article were reported.

References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

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Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  1. 1.Immunobiology and Cancer Research ProgramOklahoma Medical Research FoundationOklahoma CityUSA

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