Therapeutic molecular targets of SSc-ILD. Issue 2 (March 2020)

• Record Type: Journal Article
• Authors: Zhang, Yun; Distler, Jörg HW
• Is Part Of: Journal of scleroderma and related disorders. Volume 5:Issue 2(2020)Supplement
• Page Start: 17
• Page End: 30
• Publication Date: 2020-03
• Subjects: Systemic sclerosis -- pulmonary fibrosis -- molecular target; Scleroderma (Disease) -- Periodicals; Systemic scleroderma -- Periodicals; Fibrosis -- Periodicals; 616.544
• DOI: 10.1177/2397198319899013
• Languages: English
• ISSNs: 2397-1983

Abstract

Systemic sclerosis is a fibrosing chronic connective tissue disease of unknown etiology. A major hallmark of systemic sclerosis is the uncontrolled and persistent activation of fibroblasts, which release excessive amounts of extracellular matrix, lead to organ dysfunction, and cause high mobility and motility of patients. Systemic sclerosis–associated interstitial lung disease is one of the most common fibrotic organ manifestations in systemic sclerosis and a major cause of death. Treatment options for systemic sclerosis–associated interstitial lung disease and other fibrotic manifestations, however, remain very limited. Thus, there is a huge medical need for effective therapies that target tissue fibrosis, vascular alterations, inflammation, and autoimmune disease in systemic sclerosis–associated interstitial lung disease. In this review, we discuss data suggesting therapeutic ways to target different genes in distinct tissues/organs that contribute to the development of SSc.