Fibrosing rheumatic diseases (scleroderma, MCTD, IgG4-related disease, scleroderma mimics)
Shervin Assassi, MD, MS
University of Texas McGovern Medical School at Houston
Robert Lafyatis, MD
University of Pittsburgh
Benjamin Korman, MD
University of Rochester
Systemic sclerosis (SSc)/scleroderma is an autoimmune disease of unknown etiology that results in fibrosis of numerous end-organ tissue,s including the heart, kidney, skin, GI tract and lung through unknown mechanisms, and often results in increased mortality. Although clinically defined SSc subsets are associated with internal organ complications and death, they cannot predict disease course on a per-patient basis or inform treatment decisions. Numerous studies relate transcriptional signatures from PBMC or whole skin of SSc patients to disease activity. However, these studies still have not led to new patient stratification procedures or precision medicine approaches. Moreover, skin has been used a surrogate for disease activity in SSc patients, but the mechanism of fibrosis in the various tissues are unknown and may be different. At this session, faculty will provide updates on the mechanism for fibrosis in affected tissues from SSc patients, the utility of using skin as a surrogate in lung, heart or GI tract disease progression for clinical trials, and the uses of new genomic approaches to stratify patients.
