Journal
BEST PRACTICE & RESEARCH IN CLINICAL RHEUMATOLOGY
Volume 26, Issue 1, Pages 13-24Publisher
ELSEVIER SCI LTD
DOI: 10.1016/j.berh.2012.01.011
Keywords
Systemic sclerosis; Pathogenesis; Treatment
Categories
Ask authors/readers for more resources
Systemic sclerosis (SSc) is a multisystem disease with a variable clinical course and a poor prognosis corresponding to extent of microangiopathy and skin and internal organ fibrosis. Microvascular damage provokes immune cells to produce autoantibodies, pro-inflammatory and pro-fibrotic cytokines and chemokines. The hallmark of SSc is excessive collagen production by activated fibroblasts and myofibroblasts, and its accumulation in skin and internal organs. Better understanding of SSc pathogenesis resulted in the development of drugs, such as prostanoids, endothelin-1 and phosphodiesterase inhibitors, for treatment of pulmonary arterial hypertension and digital ulcers. The use of biological therapies and anti-fibrotic agents is under investigation. Stem cell transplantation seems to be promising in restarting the immune system to diminish fibrosis and restore microvasculature. Future research will be directed at genetic factors, diagnostic and prognostic markers for fibrosis and microangiopathy, and development of drugs directed to pathogenic key cells and mediators. (C) 2012 Elsevier Ltd. All rights reserved.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available