Vasculopathy, immune activation, and fibrosis are hallmarks in the pathogenesis of systemic sclerosis (SSc). So far, the complex interplay of vasculopathy, the cellular immune system, and fibrosis in SSc remains enigmatic. A better understanding of the pathogenesis, however, may help to develop effective targeted therapies for the treatment of SSc. Several mouse models are available to study different aspects of the disease. Because none of these models can encompass all features of human SSc, a critical selection of animal models is the basis for successful in vivo studies. Herein, we will present the most important animal models of SSc. The characteristics, advantages, and limitations of each model will be discussed and compared. The talk aims to guide the selection of suitable animal models of SSc for different research purposes.
Disclosure of Interest J. Distler Shareholder of: 4D Science, Consultant for: Actelion, Pfizer, Ergonex, BMS, Celgene, Bayer Pharma, Boehringer Ingelheim, JB Therapeutics, Sanofi-Aventis, Novartis, Array Biopharma and Active Biotech, Roche, Speakers bureau: BMS, Celgene, Bayer Pharma, Boehringer Ingelheim