IgG4相关胆管炎（IRC）是IgG4相关疾病（IgG4-RD）的主要肝胆疾病表现，属于一种系统性纤维炎性疾病。目前认为IgG4-RD和IRC的发病机制是多因素的，存在遗传易感性和环境因素，如蓝领工作是一项重要的危险因素。在IgG4-RD中已经描述了多种自身抗原，包括A11型黏附素和511-E8型层粘连蛋白，这些自身抗原通过增强分泌和屏障功能可能部分发挥保护性作用。对于最近描述的其他自身抗原，如galectin-3和prohibitin 1，其在胆管细胞中的独特作用似乎不太明显。与这些自身抗原相关联的IgG4+浆细胞克隆扩增在IRC患者中存在，并在成功治疗后消失。最近的研究显示，与IgG4-RD的发病机制有关的特异性T细胞亚型包括调节性T细胞、滤泡辅助2型T细胞、周围辅助T细胞和细胞毒性CD8+和CD4+ SLAMF7+ T细胞。IRC的临床表现常常模仿其他胆管疾病，如原发性硬化性胆管炎或胆管癌，这可能导致不合适的治疗和潜在的无效手术干预。由于缺乏特异性生物标志物，IRC的诊断根据HISORt准则的组织学、影像学、血清学、其他器官表现和治疗反应来进行。IRC的治疗旨在预防或减轻器官损伤，改善症状，包括缓解诱导、缓解维持和长期管理。对于缓解诱导，糖皮质激素效果显著，治疗后可以引入免疫调节剂作为缓解维持的替代药物。对IRC发病机制的进一步认识将有助于未来的改进诊断和新的治疗策略。版权©2023 The Author(s). Elsevier B.V.版权所有。

IgG4-related cholangitis (IRC) is the major hepatobiliary manifestation of IgG4-related disease (IgG4-RD), a systemic fibroinflammatory disorder. The pathogenesis of IgG4-RD and IRC is currently viewed as multifactorial with evidence of a genetic predisposition and also environmental factors such as blue-collar work being a major risk factor. Various autoantigens have been described in IgG4-RD including annexin A11 and laminin 511-E8, which may partially exert a protective function in cholangiocytes through enhancing secretion and barrier function, respectively. For the other recently described autoantigens, galectin-3 and prohibitin 1, a distinct role in cholangiocytes appears less apparent. In relation to these autoantigens, oligoclonal expansion of IgG4+ plasmablasts are present in IRC patients and disappear upon successful treatment. More recently, specific T cell subtypes including regulatory T cells, follicular T helper 2 cells, peripheral T helper cells and cytotoxic CD8+ and CD4+ SLAMF7+ T cells have been implicated in the pathogenesis of IgG4-RD. The clinical presentation of IRC often mimics other biliary diseases such as primary sclerosing cholangitis or cholangiocarcinoma, which may lead to inappropriate medical and potentially invalidating surgical interventions. As specific biomarkers are lacking, diagnosis is made according to the HISORt criteria comprising histopathology, imaging, serology, other organ manifestations and response to therapy. Treatment of IRC aims to prevent or alleviate organ damage and to improve symptoms and consists of (i) remission induction, (ii) remission maintenance and (iii) long-term management. For remission induction glucocorticosteroids are highly effective, after which immunomodulators can be introduced for maintenance of remission as glucocorticosteroid sparing alternatives. Increased insight into the pathogenesis of IRC will lead to improved diagnosis and novel therapeutic strategies in the future.Copyright © 2023 The Author(s). Published by Elsevier B.V. All rights reserved.