Systemic sclerosis (SSc, scleroderma) is an often-fatal disease characterized by connective tissue fibrosis of skin and internal organs. In scleroderma, there is an excessive production and accumulation of extracellular matrix (ECM) components resulting from an increase in collagen synthesis and matrix stability. Understanding how this how excessive ECM is produced and remodeled may represent a novel therapeutic approach. In this review, the transcription factors and collagen-modifying enzymes underlying collagen overexpression and enhancing stability in SSc are discussed. Moreover, the role of matrix stiffness in promoting fibrosis via a feed-forward mechanism is discussed. Indeed, the emerging evidence is that enhanced ECM remodeling resulting in increased ECM stiffness may be sufficient in itself to sustain persistence fibrosis in SSc.
WHO estimates that half of all patients worldwide are non-adherent to their prescribed medication. The consequences of poor adherence can be catastrophic, on both the individual and population level.
Join our expert panel to discover why you need to understand the drivers of non-adherence in your patients, and how you can optimize medication adherence in your clinics to drastically improve patient outcomes.
Watch Dr. Anne Marie Valente present the last year's highlights in pediatric and congenital heart disease in the official ACC.24 Year in Review session.