Emerging strategies for treatment of systemic sclerosis. Issue 2 (May 2016)

• Record Type: Journal Article
• Authors: Khanna, Dinesh; Distler, Jörg H.W.; Sandner, Peter; Distler, Oliver
• Is Part Of: Journal of scleroderma and related disorders. Volume 1:Issue 2(2016)
• Page Start: 186
• Page End: 193
• Publication Date: 2016-05
• Subjects: Fibroblast differentiation -- IL-6 -- PPAR-γ -- soluble guanylate cyclase stimulators -- TGFβ -- tyrosine kinase inhibitor; Scleroderma (Disease) -- Periodicals; Systemic scleroderma -- Periodicals; Fibrosis -- Periodicals; 616.544
• DOI: 10.5301/jsrd.5000207
• Languages: English
• ISSNs: 2397-1983

Abstract

Systemic sclerosis is a heterogeneous condition characterized by microvascular damage, dysregulation of the immune system, and progressive fibrosis affecting skin and internal organs. Currently, there are no approved disease-modifying therapies, and management mostly involves treatment of organ-specific complications. In recent years, major advances have greatly improved our understanding of the disease process, especially the molecular mechanisms by which fibrosis becomes self-sustaining. We discuss selected aspects of these mechanisms with a focus on those relevant to ongoing efforts to develop disease-modifying therapies. We also discuss advances in identification of patient subtypes, and selected examples of potential disease-modifying therapies in clinical development.