Fibroblasts are the effector cells of fibrosis characteristic of systemic sclerosis (SSc, scleroderma) and other fibrosing conditions. The excess production of extracellular matrix (ECM) proteins is the hallmark of fibrosis in different organs, such as skin and lung. Experiments designed to assess the pro-fibrotic capacity of factors, their signaling pathways, and potential inhibitors of their effects that are conducted in fibroblasts have paved the way for planning clinical trials in SSc. As such, fibroblasts have proven to be valuable tools in the search for effective anti-fibrotic therapies for fibrosis. Herein we highlight the characteristics of fibroblasts, their role in the etiology of fibrosis, utility in experimental assays, and contribution to drug development and clinical trials in SSc.
J scleroderma relat disord 2017; 2(2): 100 - 107
Article Type: REVIEW
AuthorsSara M. Garrett, DeAnna Baker Frost, Carol Feghali-Bostwick
- • Accepted on 13/04/2017
- • Available online on 19/05/2017
- • Published in print on 31/05/2017
This article is available as full text PDF.
- Garrett, Sara M. [PubMed] [Google Scholar]
- Baker Frost, DeAnna [PubMed] [Google Scholar]
- Feghali-Bostwick, Carol [PubMed] [Google Scholar] , * Corresponding Author (firstname.lastname@example.org)
Department of Medicine, Division of Rheumatology & Immunology, Medical University of South Carolina, Charleston, SC - USA