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PDGF/PDGFR: A Possible Molecular Target in Scleroderma Fibrosis
Authors:Chiara Paolini  Silvia Agarbati  Devis Benfaremo  Matteo Mozzicafreddo  Silvia Svegliati  Gianluca Moroncini
Affiliation:1.Department of Clinical and Molecular Sciences, Università Politecnica delle Marche, 60126 Ancona, Italy; (C.P.); (S.A.); (D.B.); (M.M.); (S.S.);2.Department of Internal Medicine, Clinica Medica, Ospedali Riuniti “Umberto I-G.M. Lancisi-G. Salesi”, 60126 Ancona, Italy
Abstract:Systemic sclerosis (SSc) is a clinically heterogeneous disorder of the connective tissue characterized by vascular alterations, immune/inflammatory manifestations, and organ fibrosis. SSc pathogenesis is complex and still poorly understood. Therefore, effective therapies are lacking and remain nonspecific and limited to disease symptoms. In the last few years, many molecular and cellular mediators of SSc fibrosis have been described, providing new potential options for targeted therapies. In this review: (i) we focused on the PDGF/PDGFR pathway as key signaling molecules in the development of tissue fibrosis; (ii) we highlighted the possible role of stimulatory anti-PDGFRα autoantibodies in the pathogenesis of SSc; (iii) we reported the most promising PDGF/PDGFR targeting therapies.
Keywords:platelet-derived growth factor (PDGF)  PDGF receptor (PDGFR)  fibrosis  systemic sclerosis (SSc)  scleroderma (SSc)  anti-PDGFR autoantibodies  PDGF/PDGFR targeting therapy
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