The development of new therapies targeting the three cardinal pathological processes of systemic sclerosis

2017 Fiscal Year Final Research Report

• Project/Area Number: 16K15511
• Research Category: Grant-in-Aid for Challenging Exploratory Research
• Allocation Type: Multi-year Fund
• Research Field: Collagenous pathology/Allergology
• Research Institution: The University of Tokyo
• Principal Investigator: Sato Shinichi 東京大学, 医学部附属病院, 教授 (20215792)
• Project Period (FY): 2016-04-01 – 2018-03-31
• Keywords: 全身性強皮症 / 動物モデル / 線維化 / 血管障害 / 免疫異常 / Fli1 / ブレオマイシン

Outline of Final Research Achievements

Systemic sclerosis (SSc) is a multisystem autoimmune disease chracterized by vasculopathy and fibrosis of the skin and various internal organs. We recently established a new experimental system in which the three pathological features of SSc, such as immune abnormality, vasculopathy, and tissue fibrosis, can be separately assessed by using endothelial cell-specific Fli1 knockout mice, which mimic SSc vasculopathy, and bleomycin-treated mice, which resemble inflammation and tissue fibrosis of SSc.In this study, we identified the mechanisms by which glycyrrhizin and cyclophosphamide affect the developmental process of SSc by using this experimental system. At the time of writing, glycyrrhizin is applied for a patent, and we are conducting a prospective clinical study to evaluate the efficacy of glycyrrhizin on SSc vasculopathy.

Free Research Field

膠原病、特に全身性強皮症