期刊
CURRENT OPINION IN RHEUMATOLOGY
卷 21, 期 6, 页码 649-655出版社
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/BOR.0b013e328330da9b
关键词
apoptosis; epithelial cells; fibroblasts; 3-hydroxymethylglutaryl-Coenzyme A reductase inhibitors; endothelin receptor antagonists; mesenchymal stem cells; peroxisome proliferator-activated receptor-gamma agonists; protein kinase inhibitors
类别
资金
- National Institutes of Health [U10 HLOS0510]
- Gulf South IPF Clinical Research Network [R01 HI-067967]
Purpose of review The lung in systemic sclerosis (scleroderma) is susceptible to fibrosis and the ensuing respiratory insufficiency contributes to significant morbidity and mortality in this disease. The lack of effective therapies for pulmonary fibrosis has spurred a re-evaluation of pathobiological paradigms and therapeutic strategies in scleroderma-associated interstitial lung disease and in idiopathic pulmonary fibrosis. The purpose of this review is to examine emerging new therapeutic targets that modulate pro-fibrotic phenotypes of tissue-resident cells and the associated aberrant tissue remodeling responses in fibrotic disorders. Recent findings Progressive forms of tissue fibrosis, including scleroderma, are characterized by an accumulation of activated mesenchymal cells and their secreted extracellular matrix proteins in association with dysrepair of epithelial and endothelial cells. Recent studies suggest that emergence of cellular phenotypes that perpetuate loss of cellular homeostasis is characteristic of many fibrosis-related clinical syndromes. Summary Therapeutic strategies that modulate the fate/phenotype of reparative structural cells, including epithelial, endothelial, and mesenchymal cells, offer new opportunities for the development of more effective drugs for the treatment of fibrosis.
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