Abstract
Systemic sclerosis (SSc) is a connective tissue disorder of unknown etiology characterized by the presence of multiple autoantibodies, including those against angiotensin and endothelin receptors. Patients with SSc can develop heterogeneous clinical manifestations including microvascular damage, the dysregulation of innate and adaptive immunity, and generalized fibrosis of multiple organs. Autoantibodies against angiotensin II type I receptor (AT1R) and endothelin-1 type A receptor (ETAR) play important roles in the pathogenesis of SSc. These autoantibodies regulate physiological processes ranging from production of collagen by skin fibroblasts to angiogenesis modulation. Understanding the mechanisms behind autoantibodies against AT1R and ETAR could provide insight to future novel therapies for SSc patients. In this review, we focus on elucidating the immunopathological mechanisms triggered by anti-AT1R and anti-ETAR autoantibodies to summarize current knowledge about vascular abnormalities resulting in progressive damage of organs seen in patients with SSc.
| Original language | English |
|---|---|
| Journal | Autoimmunity Reviews |
| Volume | 15 |
| Issue number | 7 |
| Pages (from-to) | 690-694 |
| Number of pages | 5 |
| ISSN | 1568-9972 |
| DOIs | |
| Publication status | Published - 01.07.2016 |
Funding
We acknowledge all the patients and their families for their participation in our studies. We thank Dr. Anja Kerstein, Dr. Antje Mueller, and Dr. Barbara Russo all from the Department of Rheumatology–University of Lübeck and Dr. Jing Sun. from the Institute for Systemic Inflammation Research–University of Lübeck for proofreading. In addition, we thank the support and funding from the intramural grant of the University of Lübeck, German Systemic Sclerosis Network (DNSS) , DFG grant RI 1056-11-1/2 , Actelion Pharmaeutical GmbH Germany, GSK, CellTrend, Scleroderma Foundation, and MirjamLichy Foundation.
Research Areas and Centers
- Academic Focus: Center for Brain, Behavior and Metabolism (CBBM)
