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This article is part of the supplement: Advances in systemic sclerosis and related fibrotic and vascular conditions

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Fibrosis in connective tissue disease: the role of the myofibroblast and fibroblast-epithelial cell interactions

Thomas Krieg1*, David Abraham2 and Robert Lafyatis3

Author Affiliations

1 Department of Dermatology, University of Köln, Kerpener Strasse, D-50924 Köln, Germany

2 Department of Medicine, Centre for Rheumatology and Connective Tissue Diseases, Royal Free and University College, Rowland Hill Street, London, NW3 2PF, UK

3 Rheumatology Department, Boston University of Medicine, Albany Street, Boston, Massachusetts 02118-2394, USA

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Arthritis Research & Therapy 2007, 9(Suppl 2):S4  doi:10.1186/ar2188

Published: 15 August 2007


Fibrosis, characterized by excessive extracellular matrix accumulation, is a common feature of many connective tissue diseases, notably scleroderma (systemic sclerosis). Experimental studies suggest that a complex network of intercellular interactions involving endothelial cells, epithelial cells, fibroblasts and immune cells, using an array of molecular mediators, drives the pathogenic events that lead to fibrosis. Transforming growth factor-β and endothelin-1, which are part of a cytokine hierarchy with connective tissue growth factor, are key mediators of fibrogenesis and are primarily responsible for the differentiation of fibroblasts toward a myofibroblast phenotype. The tight skin mouse (Tsk-1) model of cutaneous fibrosis suggests that numerous other genes may also be important.