Systemic Sclerosis Dissected: Creating an In Vitro model

Abstract

Systemic sclerosis (SSc) is an chronic immune mediated disease characterized by fibrosis of the skin (scleroderma) and internal organs. Fibrosis of these tissues may ultimately lead to organ failure, potentially leading to a fatal outcome. Due to the complex pathology of this disease there is still a need for new therapies. Many studies are performed using in vitro models to simulate the diseased tissue. While tissue engineering has evolved rapidly over the last years, these in vitro models are still limited in their representation of the scleroderma. Therefore, this review aims to summarize the most critical components of SSc as well as the development of 3D in vitro skin tissue models in order design a new in vitro model. Leukocyte associated immunoglobulin-like receptor-1 (LAIR-1) is an inhibitory receptor present on many immune cells. LAIR-1 is involved in the development of SSc through insufficient inhibition of the immune system. This review will focus particularly on including allowing cell-to-cell and cell-to-matrix contacts to allow for further research into the role of LAIR-1 in SSc.