Scleroderma is a chronic, often progressive, autoimmune disease in which the body's immune system attacks its own body. The disease, which literally means "hard skin," can cause a thickening and tightening of the skin. In some cases it causes serious damage to internal organs including the lungs, heart, kidneys, esophagus and gastrointestinal tract. Some medicines and treatments can help with certain symptoms, but there is still no cure for scleroderma, which affects about 300,000 nationwide. (By way of comparison, about 50,000 people have muscular dystrophy, 250,000 are estimated to have lupus and 350,000 have multiple sclerosis.)
Supporting actors sometimes steal the show. In a new study published today in Cell, researchers headed by Prof. Ido Amit at the Weizmann Institute of Science have shown that supporting cells called fibroblasts, long viewed as uniform background players, are in fact extremely varied and vital.
A research team at the Medical University of South Carolina led by Carol Feghali-Bostwick, Ph.D., reports in the Journal of Clinical Investigation Insight that the E4 peptide reverses fibrosis, or scarring, in human and mouse tissues by activating an antifibrotic pathway that is common to all organ systems.
Severe injuries to the lung from diseases such as COVID-19 trigger abnormal stem cell repair that alters the architecture of the lung. The aberrant stem cell differentiation in response to injury can prevent the restoration of normal lung function.
Systemic sclerosis is an autoimmune disease associated with inflammation and fibrosis, or scarring, that affects organs including the skin, heart, kidney and lungs.