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TGFβ—a role in systemic sclerosis?
Author(s) -
Cotton Shirley A.,
Herrick Ariane L.,
Jayson Malcolm I. V.,
Freemont Anthony J.
Publication year - 1998
Publication title -
the journal of pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.964
H-Index - 184
eISSN - 1096-9896
pISSN - 0022-3417
DOI - 10.1002/(sici)1096-9896(199801)184:1<4::aid-path968>3.0.co;2-0
Subject(s) - fibroblast , fibrosis , fibronectin , transforming growth factor , connective tissue , pathogenesis , transforming growth factor beta , immunology , stimulation , scleroderma (fungus) , connective tissue disease , medicine , biology , pathology , extracellular matrix , cancer research , in vitro , microbiology and biotechnology , endocrinology , autoimmune disease , antibody , biochemistry , inoculation
Systemic sclerosis (SSc) is a multisystem connective tissue disorder in which there is progressive fibrosis. Transforming growth factor beta (TGFβ) has wide‐ranging cellular actions. It is a potent chemoattractant for human dermal fibroblasts, from which it may induce synthesis of collagen, which suggests that it may have a central role to play in the pathogenesis of SSc. This is supported to some extent by in vitro studies. SSc fibroblasts produce more collagens and fibronectin than normal fibroblasts and elevated TIMP levels have been observed, all of which could be explained on the basis of TGFβ stimulation of fibroblasts. Some studies have suggested that fibroblasts are the source of TGFβ. However, the serum of patients with SSc is cytotoxic to endothelial cells, which could culminate in TGFβ synthesis by them, with secondary fibroblast stimulation. The role of TGFβ remains elusive, although it would seem an ideal candidate as a mediator of fibrosis in systemic sclerosis. © 1998 John Wiley & Sons, Ltd.