Role of cytokines in scleroderma: Use of animal models

Scleroderma is a fibrotic condition characterized by immunological abnormalities, vascular injury, and increased accumulation of matrix proteins in the skin. Although the etiology of scleroderma has not been fully elucidated, a growing body of evidence suggests that the overproduction of extracellular matrix by activated fibroblasts results from complex interactions among endothelial cells, lymphocytes, macrophages, and fibroblasts, via a number of mediators.

Cytokines, chemokines, and growth factors secreted by inflammatory cells and mesenchymal cells (fibroblasts and myofibroblasts) play an important role in the fibrotic process of scleroderma. Animal models are important tools for achieving a better understanding of the pathophysiology of scleroderma. In this review, we summarize some of the current insights into the role of cytokines in human as well as animal models of scleroderma.