Reversing lung fibrosis in scleroderma requires an increase in antifibrotic proteins

Much of the research on scleroderma, a connective tissue disease that causes scarring, or fibrosis, has focused on the increased number of proteins promoting fibrosis in these patients. A Medical University of South Carolina (MUSC) research team took a different tact and measured the levels of an antifibrotic protein, Cathepsin L, in these patients. They report in Rheumatology that patients with scleroderma had reduced levels of this antifibrotic protein, and the Cathepsin L that they did have was packaged in an inactive state that deprived it of its antifibrotic function. The team was led by Carol Feghali-Bostwick, Ph.D., the Kitty Trask Holt Endowed Chair for Scleroderma Research, and M.D.-Ph.D. student Joe Mouawad, a National Scleroderma Foundation predoctoral fellowship awardee.