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. 2011 Jun;5(2):125-9.
doi: 10.1007/s12079-011-0122-6. Epub 2011 Jan 29.

Possible strategies for anti-fibrotic drug intervention in scleroderma

Andrew Leask  1
Affiliations

Possible strategies for anti-fibrotic drug intervention in scleroderma

Andrew Leask. J Cell Commun Signal. 2011 Jun.

Abstract

There are no approved drugs for treating the fibrosis in scleroderma (systemic sclerosis, SSc). Myfibroblasts within connective tissue express the highly contractile protein α-smooth muscle actin (α-SMA) and are responsible for the excessive synthesis and remodeling of extracellular matrix (ECM) characterizing SSc. Drugs targeting myofibroblast differentiation, recruitment and activity are currently under consideration as anti-fibrotic treatments in SSc but thus far have principally focused on the transforming growth factor β (TGFβ), endothelin-1 (ET-1), connective tissue growth factor (CCN2/CTGF) and platelet derived growth factor (PDGF) pathways, which display substantial signaling crosstalk. Moreover, peroxisome proliferator-activated receptor (PPAR)γ also appears to act by intervening in TGFβ signaling. This review discusses these potential candidates for antifibrotic therapy in SSc.

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