Masoumi Maryam, Bodaghi Ali Bayat, Khorramdelazad Hossein, Ebadi Erfan, Houshmandfar Sheyda, Saeedi-Boroujeni Ali, Karami Jafar
Clinical Research Development Unit, Shahid Beheshti Hospital, Qom University of Medical Sciences, Qom, Iran.
Student Research Committee, Khomein University of Medical Sciences, Khomein, Iran.
Heliyon. 2024 Jul 31;10(15):e35445. doi: 10.1016/j.heliyon.2024.e35445. eCollection 2024 Aug 15.
The article delves into the pathogenesis of systemic sclerosis (SSc) with an emphasis on immunometabolism dysfunctions. SSc is a complex autoimmune connective tissue disorder with skin and organ fibrosis manifestation, vasculopathy, and immune dysregulation. A growing amount of research indicates that immunometabolism plays a significant role in the pathogenesis of autoimmune diseases, including SSc. The review explores the intricate interplay between immune dysfunction and metabolic alterations, focusing on the metabolism of glucose, lipids, amino acids, the TCA (tricarboxylic acid) cycle, and oxidative stress in SSc disease. According to recent research, there are changes in various metabolic pathways that could trigger or perpetuate the SSc disease. Glycolysis and TCA pathways play a pivotal role in SSc pathogenesis through inducing fibrosis. Dysregulated fatty acid β-oxidation (FAO) and consequent lipid metabolism result in dysregulated extracellular matrix (ECM) breakdown and fibrosis induction. The altered metabolism of amino acids can significantly be involved in SSc pathogenesis through various mechanisms. Reactive oxygen species (ROS) production has a crucial role in tissue damage in SSc patients. Indeed, immunometabolism involvement in SSc is highlighted, which offers potential therapeutic avenues. The article underscores the need for comprehensive studies to unravel the multifaceted mechanisms driving SSc pathogenesis and progression.
本文深入探讨了系统性硬化症(SSc)的发病机制,重点关注免疫代谢功能障碍。SSc是一种复杂的自身免疫性结缔组织疾病,表现为皮肤和器官纤维化、血管病变以及免疫失调。越来越多的研究表明,免疫代谢在包括SSc在内的自身免疫性疾病的发病机制中起着重要作用。该综述探讨了免疫功能障碍与代谢改变之间的复杂相互作用,重点关注SSc疾病中葡萄糖、脂质、氨基酸的代谢、三羧酸(TCA)循环以及氧化应激。根据最近的研究,各种代谢途径的变化可能引发或延续SSc疾病。糖酵解和TCA途径通过诱导纤维化在SSc发病机制中起关键作用。脂肪酸β-氧化(FAO)失调及随之而来的脂质代谢导致细胞外基质(ECM)分解失调和纤维化诱导。氨基酸代谢的改变可通过多种机制显著参与SSc发病机制。活性氧(ROS)的产生在SSc患者的组织损伤中起关键作用。事实上,免疫代谢在SSc中的参与得到了强调,这为潜在的治疗途径提供了方向。本文强调需要进行全面研究,以揭示驱动SSc发病机制和进展的多方面机制。