Journal
JOINT BONE SPINE
Volume 80, Issue 1, Pages 23-28Publisher
ELSEVIER FRANCE-EDITIONS SCIENTIFIQUES MEDICALES ELSEVIER
DOI: 10.1016/j.jbspin.2012.06.005
Keywords
Systemic sclerosis; Animal models; Dermal fibrosis
Categories
Ask authors/readers for more resources
Vasculopathy, immunological abnormalities, and fibrosis are the key features in the pathogenesis of systemic sclerosis (SSc). Expression of each of the three pathologic features varies among SSc patients leading to disease heterogeneity and variable organ manifestations. Although the etiology of SSc has not yet been fully elucidated, a growing body of evidence suggests that extracellular matrix overproduction by activated fibroblasts results from a complex interplay between endothelial cells, immune cells and fibroblasts through cell cell and cell matrix interactions and communications. Relevant animal models are essential tools to in-depth investigate pathogenesis of SSc. Several murine and avian models are available; however, some models display inflammation followed by fibrosis, whether some others primarily mimic autonomous fibroblast activation. In addition, typical microvascular changes of SSc are only observed in few models. Therefore, none of these animal models encompasses all features of the human disease and a critical selection is mandatory for successful in vivo studies. Hence, we will provide an overview of the most important experimental models of dermal fibrosis and SSc and discuss their respective contribution to the better understanding of SSc pathogenesis. (c) 2012 Societe francaise de rhumatologie. Published by Elsevier Masson SAS. All rights reserved.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available