生发中心
生物
免疫学
淋巴瘤
计算生物学
自身免疫
信号转导
免疫系统
癌症研究
自身免疫性疾病
生物信息学
断点群集区域
细胞生物学
抗体
CXCL13型
神经科学
受体
否定选择
干扰素
免疫疗法
疾病
医学
精密医学
转录因子
作者
Loukas G. Chatzis,Dimitris Anastasios Palamidas,Thomas Ernst Dorner
标识
DOI:10.1016/j.coi.2025.102712
摘要
Sjögren's disease (SjD) is a chronic autoimmune disorder in which sustained B-cell activation drives glandular injury and systemic complications. Epithelial stress and interferon tone amplify B-cell activating factor (BAFF)-dependent survival, skewing selection toward autoreactive clones in both glands and blood. In addition, single-cell B-cell receptor analyses have uncovered interferon-high endotypes with tissue-imprinted oligoclonality and biased isotype and light-chain usage. Within salivary glands, ectopic germinal centers and FcRL4⁺ B cells act as local 'training sites,' integrating Tfh/Tph help, CXCL13 cues, and BAFF/APRIL-NF-κB signaling to sustain plasmablast differentiation. Extrafollicular trajectories - double-negative and age-associated B cells - expand in IFN/TLR7 and IL-21 milieus, while regulatory B-cell restraint is diminished. Emerging data also implicate glycolysis and mTORC1-GLUT1 metabolism in sustaining B-cell hyperactivation. Chronic B-cell receptor signaling and clonal evolution provide a bridge to lymphoma risk. In this review, we outline how these converging pathways define molecular endotypes and propose a precision framework linking them to targeted therapy in SjD.
科研通智能强力驱动
Strongly Powered by AbleSci AI