Systemic sclerosis is a serve autoimmune connective tissue disease, which is characterized by autoimmunity, vasculopathy and fibrosis. The pathogenesis of the development of SSc is only partially known. Animal models, particularly mouse models are powerful research tools helping us to understand disease pathogenesis. So far, more than ten mouse models for SSc have been established, including both spontaneous and induced models. However, none of them is humanized mouse model. Although both human and mice are mammals, there are still considerable differences between the two species, making a gap between mouse models and human autoimmune diseases. Therefore, humanized mouse model is invaluable tool to fill the gap between human autoimmune diseases and their mouse models.

Recently, we generated a novel humanized mouse model for SSc by transfer of PMBC from patients into immune deficient mice. After transfer of PMBC from SSc patients, mice develop a SSc-like disease symptoms, including generation of anti-nuclear antibodies and inflammation in multiple inner organs, while mice received PMBC from healthy donors do not.

Therefore, we aim to further characterize this novel humanized mouse model for SSc and to investigate the role of T- and B-cells in the pathogenesis in this humanized mouse model. Further investigation of this novel humanized mouse model will help us to understand the pathogenesis of SSc. Furthermore, this novel humanized model represents a individualized model for SSc, which will provide some hints for personalized medicine.