IgG4相关硬化性胆管炎药物治疗新进展

摘要/Abstract

摘要： IgG₄相关硬化性胆管炎(IgG₄-SC)是IgG₄相关疾病(IgG₄-RD)在胆道的主要表现,常与IgG₄相关自身免疫性胰腺炎(IgG₄-AIP)共存,特征性表现为胆管壁弥漫性增厚、“节段性”狭窄及大量IgG₄阳性浆细胞浸润。其治疗以药物干预为主,分为诱导缓解和维持治疗两个阶段。糖皮质激素仍为一线治疗,诱导缓解率可达95%以上,但复发率高,长期维持治疗可降低复发及胆管癌风险。免疫抑制剂(如硫唑嘌呤、吗替麦考酚酯、来氟米特等)可辅助激素减量并延缓复发。生物制剂,特别是B细胞靶向治疗(如利妥昔单抗、伊奈利珠单抗),在难治性IgG₄-SC及激素不耐受患者中显示出良好的效果。

曾馨, 段维佳, 贾继东. IgG₄相关硬化性胆管炎药物治疗新进展[J]. 肝脏, 2025, 30(9): 1174-1177.

/ 推荐

参考文献

[1] 费允云,刘燕鹰,董凌莉,等. IgG₄相关性疾病诊疗规范[J]. 中华内科杂志, 2023, 62(10):1161-1171.
[2] Wallace Z S, Miles G, Smolkina E, et al. Incidence, prevalence and mortality of IgG₄-related disease in the USA: a claims-based analysis of commercially insured adults [J]. Ann Rheum Dis, 2023, 82(7):957-962.
[3] Kamisawa T, Nakazawa T, Tazuma S, et al. Clinical practice guidelines for IgG₄-related sclerosing cholangitis [J]. J Hepatobiliary Pancreat Sci, 2019, 26(1):9-42.
[4] Nakazawa T, Kamisawa T, Okazaki K, et al. Clinical diagnostic criteria for IgG₄-related sclerosing cholangitis 2020: (Revision of the clinical diagnostic criteria for IgG₄-related sclerosing cholangitis 2012) [J]. J Hepatobiliary Pancreat Sci, 2021, 28(3):235-242.
[5] European Association for the Study of the L. EASL Clinical Practice Guidelines on sclerosing cholangitis [J]. J Hepatol, 2022, 77(3):761-806.
[6] Wu Q, Chang J, Chen H, et al. Efficacy between high and medium doses of glucocorticoid therapy in remission induction of IgG₄-related diseases: a preliminary randomized controlled trial [J]. Int J Rheum Dis, 2017, 20(5):639-646.
[7] Iwasaki S, Kamisawa T, Koizumi S, et al. Assessment in steroid trial for IgG₄-related sclerosing cholangitis [J]. Adv Med Sci, 2015, 60(2):211-215.
[8] Kubota K, Kamisawa T, Nakazawa T, et al. Steroid therapy still plays a crucial role and could serve as a bridge to the next promising treatments in patients with IgG₄-related sclerosing cholangitis: results of a Japanese Nationwide Study [J]. J Hepatobiliary Pancreat Sci, 2022.
[9] Wallace Z S, Naden R P, Chari S, et al. The 2019 American college of rheumatology/European league against rheumatism classification criteria for IgG₄-related disease [J]. Arthritis Rheumatol, 2020, 72(1):7-19.
[10] Lanzillotta M, Mancuso G, Della-Torre E. Advances in the diagnosis and management of IgG₄ related disease [J]. BMJ, 2020, 369:m1067.
[11] Culver E L, Sadler R, Bateman A C, et al. Increases in IgE, eosinophils, and mast cells can be used in diagnosis and to predict relapse of IgG₄-related disease [J]. Clin Gastroenterol Hepatol, 2017, 15(9):1444-1452 e1446.
[12] An W, Wu Z, Li M, et al. Clinical characteristics and therapeutic response of immunoglobulin G4-related disease: a retrospective study of 127 Chinese patients [J]. Orphanet J Rare Dis, 2022, 17(1):307.
[13] Kubota K, Kamisawa T, Okazaki K, et al. Low-dose maintenance steroid treatment could reduce the relapse rate in patients with type 1 autoimmune pancreatitis: a long-term Japanese multicenter analysis of 510 patients [J]. J Gastroenterol, 2017, 52(8):955-964.
[14] Kubota K, Kamisawa T, Nakazawa T, et al. Reducing relapse through maintenance steroid treatment can decrease the cancer risk in patients with IgG₄-sclerosing cholangitis: based on a Japanese nationwide study [J]. J Gastroenterol Hepatol, 2023, 38(4):556-564.
[15] Omar D, Chen Y, Cong Y,et al. Glucocorticoids and steroid sparing medications monotherapies or in combination for IgG₄-RD: a systematic review and network meta-analysis [J]. Rheumatology (Oxford), 2020, 59(4):718-726.
[16] Peng L, Nie Y, Zhou J, et al. Withdrawal of immunosuppressants and low-dose steroids in patients with stable IgG₄-RD (WInS IgG₄-RD): an investigator-initiated, multicentre, open-label, randomised controlled trial [J]. Ann Rheum Dis, 2024, 83(5):651-660.
[17] Yunyun F, Yu P, Panpan Z, et al. Efficacy and safety of low dose mycophenolate mofetil treatment for immunoglobulin G4-related disease: a randomized clinical trial [J]. Rheumatology (Oxford), 2019, 58(1):52-60.
[18] Masaki Y, Nakase H, Tsuji Y, et al. The clinical efficacy of azathioprine as maintenance treatment for autoimmune pancreatitis: a systematic review and meta-analysis [J]. J Gastroenterol, 2021, 56(10):869-880.
[19] Wang Y, Zhao Z, Gao D, et al. Additive effect of leflunomide and glucocorticoids compared with glucocorticoids monotherapy in preventing relapse of IgG₄-related disease: a randomized clinical trial [J]. Semin Arthritis Rheum, 2020, 50(6):1513-1520.
[20] Zhang P, Gong Y, Liu Z, et al. Efficacy and safety of iguratimod plus corticosteroid as bridge therapy in treating mild IgG₄-related diseases: a prospective clinical trial [J]. Int J Rheum Dis, 2019, 22(8):1479-1488.
[21] Yunyun F, Yu C, Panpan Z, et al. Efficacy of cyclophosphamide treatment for immunoglobulin G4-related disease with addition of glucocorticoids [J]. Sci Rep, 2017, 7(1):6195.
[22] Liu Y, Jin K, Yang Y, et al. Efficacy and safety of rituximab induction therapy and effect of rituximab maintenance for IgG₄-related disease: a systematic review and meta-analysis [J]. Eur J Intern Med, 2024, 127:63-73.
[23] Majumder S, Mohapatra S, Lennon R J, et al. Rituximab maintenance therapy reduces rate of relapse of pancreaticobiliary immunoglobulin G4-related disease [J]. Clin Gastroenterol Hepatol, 2018, 16(12):1947-1953.
[24] Stone J H, Khosroshahi A, Zhang W, et al. Inebilizumab for treatment of IgG₄-related disease [J]. N Engl J Med, 2025, 392(12):1168-1177.
[25] Iwamoto M, Asashima H, Sugita T, et al. An overlapping case of IgG₄-related disease and systemic lupus erythematosus treated with belimumab: a case-based review [J]. Rheumatol Int, 2024, 44(3):549-556.
[26] Matza M A, Perugino C A, Harvey L, et al. Abatacept in IgG₄-related disease: a prospective, open-label, single-arm, single-centre, proof-of-concept study [J]. Lancet Rheumatol, 2022, 4(2):e105-e112.
[27] Kanda M, Kamekura R, Sugawara M, et al. IgG₄-related disease administered dupilumab: case series and review of the literature [J]. RMD Open, 2023, 9(1).

IgG₄相关硬化性胆管炎药物治疗新进展

PDF (PC)

可视化

摘要/Abstract

引用本文

使用本文

参考文献

相关文章 15

编辑推荐

Metrics

本文评价

[1]	周兴念, 赵鹏, 高婷, 张权, 李宏. 芦可替尼联合人工肝治疗糖皮质激素应答不佳的重度肝脏移植物抗宿主病3例[J]. 肝脏, 2025, 30(2): 240-244.
[2]	邓茹心, 雷思贤, 孟忠吉. 糖皮质激素改善慢加急性肝衰竭前期及早期患者结局[J]. 肝脏, 2025, 30(1): 31-36.
[3]	何萌, 韩笑, 赵新颜. 肉芽肿性肝炎的临床-病理特点及预后分析[J]. 肝脏, 2025, 30(1): 101-106.
[4]	何萌, 韩笑, 贾继东, 赵新颜, 李鹏. 27例嗜酸性粒细胞胃肠炎临床特征分析[J]. 肝脏, 2024, 29(7): 844-847.
[5]	何泽平, 宋方彬, 徐军明. 肝移植的新进展与挑战[J]. 肝脏, 2023, 28(9): 1015-1017.
[6]	朱云, 邹正升. 糖皮质激素在药物性肝损伤应用中的争鸣[J]. 肝脏, 2023, 28(3): 273-274.
[7]	陈文雅, 赵红, 谢雯. 免疫抑制疗法相关的HBV再激活及其预防[J]. 肝脏, 2023, 28(3): 275-278.
[8]	葛文俊, 刘军, 任俊. 122例急、慢性药物性肝损伤患者临床观察[J]. 肝脏, 2023, 28(3): 343-345.
[9]	张从玉, 姜雪强. 艾米替诺福韦治疗糖皮质激素诱导HBV再激活相关肝衰竭前期1例[J]. 肝脏, 2023, 28(11): 1387-1389.
[10]	李丽, 何宇, 刘锴, 王萍. GITR阳性的效应T淋巴细胞参与AAF/PH小鼠肝脏再生调控[J]. 肝脏, 2023, 28(1): 112-116.
[11]	李涛, 王磊. 糖皮质激素治疗免疫检查点抑制剂介导的肝损伤的剂量争议[J]. 肝脏, 2022, 27(6): 615-617.
[12]	丁鹏, 仲海峰. 双重血浆分子吸附联合糖皮质激素治疗重度急性药物性肝损伤的近期疗效观察[J]. 肝脏, 2022, 27(11): 1198-1202.
[13]	刘玉, 邹颖, 张正国, 郭红英, 梅雪, 王介非, 钱志平. 糖皮质激素成功救治急性肝衰竭1例[J]. 肝脏, 2021, 26(8): 945-946.
[14]	李文庭, 杨亮, 何宏亮, 赵宗豪, 李宜. 药物性肝损伤糖皮质激素应用的时机与指征[J]. 肝脏, 2020, 25(7): 676-678.
[15]	何莹, 韩鑫, 蔺咏梅, 皇甫彤. 糖皮质激素联合抗病毒治疗HBV相关早期慢加急性肝衰竭的价值[J]. 肝脏, 2020, 25(6): 643-644.