Chiara Paolini, Silvia Agarbati, Devis Benfaremo, Matteo Mozzicafreddo, Silvia Svegliati, Gianluca Moroncini
Academic Editors: Maria Letizia Trincavelli, Claudia Martini and Diego La Mendola
Int. J. Mol. Sci.2022, 23(7), 3904; Link
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.