Pathophysiology of systemic sclerosis (scleroderma)

Kaohsiung Journal of Medical Sciences

Ann‐Helen Rosendahl¹  Katrin Schönborn¹  Thomas Krieg¹ 

[1] Translational Matrix Biology University of Cologne, Medical Faculty Cologne Germany;

关键词: extracellular matrix;    fibroblast;    fibrosis;    inflammation;   

DOI  :  10.1002/kjm2.12505

来源: DOAJ

【 摘 要 】

Abstract Systemic sclerosis (scleroderma) is an autoimmune‐triggered chronic fibrosing disease that affects the skin and many other organs. Its pathophysiology is complex and involves an early endothelial damage, an inflammatory infiltrate and a resulting fibrotic reaction. Based on a predisposing genetic background, an altered balance of the acquired and the innate immune system leads to the release of many cytokines and chemokines as well as autoantibodies, which induce the activation of fibroblasts with the formation of myofibroblasts and the deposition of a stiff and rigid connective tissue. A curative treatment is still not available but remarkable progress has been made in the management of organ complications. In addition, several breakthroughs in the pathophysiology have led to new therapeutic concepts. Based on these, many new compounds have been developed during the last years, which target these different pathways and offer specific therapeutic approaches.

【 授权许可】

Unknown