An intricate relationship between fibrosis and autoimmune diseases: a specific focus on unraveling its molecular, immunological, and epigenetic drivers

纤维化与自身免疫性疾病之间错综复杂的关系:重点揭示其分子、免疫和表观遗传驱动因素

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Abstract

Fibrosis is a pathological process of wound healing, characterized by excessive deposition of extracellular matrix (ECM) and chronic activation of fibroblasts, leading to organ scarring and a decline in function. Fibrogenesis is predominantly initiated by tissue injury and sustained inflammation, further driven by the complex interplay of growth factors, cytokines, metabolic alterations, and epigenetic reprogramming. Activated myofibroblasts and immune cells function as primary profibrotic mediators. The central molecular pathways implicated include TGF-β/SMAD signaling, non-canonical cascades such as RAS-ERK and PI3K-AKT-mTOR, and integrin-mediated mechanotransduction. These pathways collectively contribute to matrix disruption by upregulating α-SMA, collagen, lysyl oxidase, and tissue inhibitors of metalloproteinases (TIMPs). Alterations in noncoding RNAs and histone/DNA modifications stabilize genes responsible for pro-fibrotic pathways, whereas metabolic reprogramming sustains myofibblast activity. These mechanisms contribute to fibrosis in several autoimmune disorders, including rheumatoid arthritis, multiple sclerosis, Sjögren's disease, and Crohn's disease. In these conditions, persistent immune activation drives continuous crosstalk between immune cells and stromal fibroblasts, sustaining cytokine signaling and ECM remodeling. Current therapeutic approaches primarily aim to halt disease progression; however, achieving true reversal remains challenging. The significant morbidity and mortality associated with fibrotic diseases underscore the need for clinically validated biomarkers to guide effective combined therapeutic regimens capable of reversing established scarring. In this review, we explored emerging strategies that emphasize the integration of immune modulation, epigenetic reprogramming, and mechanobiological interventions to inhibit myofibroblast proliferation and facilitate matrix degradation and tissue regeneration.

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