Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties, Rheumatology Section, University of Palermo, 90133 Palermo, Italy.
Unit of Internal Medicine and Stroke, Department of Health Promotion, Maternal and Child Care, Internal Medicine and Specialized Medicine, University of Palermo, 90133 Palermo, Italy.
Cells. 2024 Feb 1;13(3):271. doi: 10.3390/cells13030271.
Large-vessel vasculitis (LVV) are autoimmune and autoinflammatory diseases focused on vascular inflammation. The central core of the intricate immunological and molecular network resides in the disruption of the "privileged immune state" of the arterial wall. The outbreak, initially primed by dendritic cells (DC), is then continuously powered in a feed-forward loop by the intimate cooperation between innate and adaptive immunity. If the role of adaptive immunity has been largely elucidated, knowledge of the critical function of innate immunity in LVV is still fragile. A growing body of evidence has strengthened the active role of innate immunity players and their key signaling pathways in orchestrating the complex pathomechanisms underlying LVV. Besides DC, macrophages are crucial culprits in LVV development and participate across all phases of vascular inflammation, culminating in vessel wall remodeling. In recent years, the variety of potential pathogenic actors has expanded to include neutrophils, mast cells, and soluble mediators, including the complement system. Interestingly, new insights have recently linked the inflammasome to vascular inflammation, paving the way for its potential pathogenic role in LVV. Overall, these observations encourage a new conceptual approach that includes a more in-depth study of innate immunity pathways in LVV to guide future targeted therapies.
巨细胞动脉炎（Large-vessel vasculitis，LVV）是一种以血管炎症为特征的自身免疫和自身炎症性疾病。错综复杂的免疫和分子网络的核心在于动脉壁“特权免疫状态”的破坏。最初由树突状细胞（Dendritic cells，DC）引发的爆发，然后通过先天免疫和适应性免疫之间的紧密合作，在正反馈环中不断得到加强。虽然适应性免疫的作用已经被广泛阐明，但先天免疫在 LVV 中的关键作用的知识仍然很脆弱。越来越多的证据表明，先天免疫参与者及其关键信号通路在调节 LVV 复杂发病机制中起着积极作用。除了 DC，巨噬细胞是 LVV 发展的关键罪魁祸首，它们参与血管炎症的所有阶段，最终导致血管壁重塑。近年来，潜在的致病因素的种类已经扩大到包括中性粒细胞、肥大细胞和可溶性介质，包括补体系统。有趣的是，最近的新见解将炎症小体与血管炎症联系起来，为其在 LVV 中的潜在致病作用铺平了道路。总的来说，这些观察结果鼓励采用一种新的概念方法，即更深入地研究 LVV 中的先天免疫途径，以指导未来的靶向治疗。
