Progress in Understanding and Treating Severe Fibrotic Diseases

Despite some new treatments in recent years, diseases characterized by extreme fibrosis continue to have poor prognoses. Two new reports raise hope that treatment progress will be forthcoming.

One team found evidence that a particular protein (c-JUN) is produced in high amounts in end-stage fibrotic diseases of many organs, including idiopathic pulmonary fibrosis, scleroderma, hepatic fibrosis, and myelofibrosis, in both mice and humans. In mice, ongoing fibrosis was dependent on sustained production of this protein. c-JUN converts cells into pathologic fibroblasts, stimulates fibroblast proliferation, and causes the wayward fibroblasts to produce a molecule that protects them from destruction by macrophages. Thus, c-JUN is an attractive tar…