Papers of The 4th Japan Scar Workshop

7. Future Therapeutic Strategies for Systemic Sclerosis

Masataka Kuwana
Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan

Systemic sclerosis (or scleroderma; SSc) is a connective tissue disease characterized by excessive fibrosis of the dermis and various internal organs, microvascular abnormalities, and immune dysregulation such as antinuclear antibody production. SSc is still an intractable disease with a 10-year survival rate < 80%, since there is currently no therapeutic agent shown to be effective for SSc by clinical trials. For instance, recent clinical trials on relaxin and fully humanized anti-TGF1 antibody that theoretically inhibits fibroblast activation ended up with negative results. Our efforts to find novel therapeutic targets focus on immune responses and angiogenesis/vasculogenesis, which are involved in the pathogenic processes of SSc. Current potential targets include intracellular tyrosine kinases; immune cells, such as monocytes, and T and B cells; and circulating progenitors, including mesenchymal stem cells and endothelial progenitor cells. Information obtained from these studies on SSc patients would be useful in developing novel therapeutic agents for organ-specific fibrotic diseases, such as interstitial lung disease, liver cirrhosis, atherosclerosis, and keloids.
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