This article appears in the following Rheumatology issue: Update in systemic sclerosis [View the issue table of contents]
Role of PDGF in fibrotic diseases and systemic sclerosis
1Division of Rheumatology and Immunology, Medical University of South Carolina, Charleston, SC, USA.
Correspondence to: M. Trojanowska, Division of Rheumatology and Immunology, Medical University of South Carolina, Charleston, SC 29425, USA. E-mail: trojanme{at}musc.edu
| Abstract |
|---|
PDGF functions as a primary mitogen and chemoattractant for cells of mesenchymal origin. Members of the PDGF family play an important role during embryonic development and contribute to the maintenance of connective tissue in adults. Deregulation of PDGF signalling has been linked to atherosclerosis, pulmonary hypertension and organ fibrosis. Elevated expression of PDGF and its receptors has been found in scleroderma skin and lung tissues. There is evidence for a TGF-β and IL-1
-dependent autocrine PDGF-A/PDGFR
signalling loop in scleroderma skin and lung fibroblasts, suggesting that a cross-talk between TGF-β and PDGF pathways may regulate chronic fibrosis in scleroderma.
KEY WORDS: Platelet-derived growth factor, Platelet-derived growth factor receptor, Transforming growth factor-β, Fibrosis, Pulmonary arterial hypertension, Scleroderma, Myofibroblast
Submitted 30 April 2008; Accepted 18 June 2008