Evaluation of fibrosis and anti-fibrotic therapeutics in systemic sclerosis
OA Version
Citation
Abstract
Systemic sclerosis (SSc) is a complex yet elusive connective tissue disease where vascular, immune, and fibrotic alterations occur. Nearly all organ systems are affected, leading to significant impacts on patients’ quality of life. A literature review was conducted to assess SSc pathogenesis and treatment options, focusing on diffuse cutaneous SSc. Vasculopathy and immune dysregulation culminate in the activation of fibroblasts into myofibroblasts. Fibrosis is the most lethal complication of SSc and is characterized by overaccumulation of extracellular matrix. Pathophysiology of skin fibrosis and lung fibrosis—comprising Idiopathic Pulmonary Fibrosis and Interstitial Lung Disease (ILD)—was examined. Fibrotic entities are generally conserved across phenotypes of fibrosis, yet some significant differences exist among the different patterns. Progressive ILD is the leading cause of death in SSc. There is an established association between decline in forced vital capacity and mortality. Though many current therapies target organ-related complications of SSc, treatments targeting fibrosis are limited. At present, there is no disease-modifying therapy for SSc. Therapies inhibiting fibrotic mechanisms were evaluated and were concluded to show promise for the treatment of SSc.
Description
2025