Clinical features, diagnosis, and treatment of IgG4-associated sclerosing cholangitis

Li LI; Hui LIU; Wenyan SONG

doi:10.3969/j.issn.1001-5256.2021.08.026

Volume 37 Issue 8

Aug. 2021

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Article Navigation > Journal of Clinical Hepatology > 2021 > 37(8): 1883-1887

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Clinical features, diagnosis, and treatment of IgG4-associated sclerosing cholangitis

DOI: 10.3969/j.issn.1001-5256.2021.08.026

Li LI^a,
Hui LIU^b,
Wenyan SONG^{c
,
,}

a.
Integrated Traditional Chinese and Western Medicine Treatment Center, Beijing YouAn Hospital, Capital Medical University, Beijing 100069, China
b.
Department of Pathology, Beijing YouAn Hospital, Capital Medical University, Beijing 100069, China
c.
Department of Radiology, Beijing YouAn Hospital, Capital Medical University, Beijing 100069, China

Research funding:

Major Scientific and Technological Projects for Prevention and Treatment of Major Infectious Diseases Such as AIDS and Viral Hepatitis (2018ZX10725504);

Key Medical Professional Projects of "Sailing Plan" (ZYLX201819)

Received Date: 2020-12-27
Accepted Date: 2021-01-20
Published Date: 2021-08-20

Abstract

Abstract

Objective To investigate the clinical features, diagnosis, and treatment of IgG4-associated sclerosing cholangitis (IgG4-SC). Methods A retrospective analysis was performed for the clinical data of 25 patients who were diagnosed with IgG4-SC in Beijing YouAn Hospital, Capital Medical University, from January 2014 to September 2020, and an electronic medical record system was used to collect general information, medical history, diagnosis and treatment processes, laboratory examination, imaging data, and pathological data. Clinical features, process of confirmed diagnosis, and cause of misdiagnosis were analyzed. Normally distributed continuous data were expressed as mean±SD, and the non-normally distributed continuous data were expressed as M(P_{minimum value}-P_{maximum value}). Results Among the 25 patients with IgG4-SC, there were 21 (84%) male patients, and the mean age was 57.61±9.73 years. Common initial symptoms included jaundice and/or yellow urine in 18 patients (78.26%) and weakness in 9 patients (39.13%). As for imaging classification, 16 patients (64%) had type Ⅰ IgG4-SC, no patient had type Ⅱa IgG4-SC, 4 (16%) had IIb IgG4-SC, 1 (4%) had type Ⅲ IgG4-SC, and 4 (16%) had type Ⅳ IgG4-SC. Pancreatic involvement was observed in 22 patients (88%). Glucocorticoids were the main treatment method, and total bilirubin achieved a mean reduction of 67.48% within 2 weeks. Among the 22 patients with a confirmed diagnosis in our hospital, 15 (68.18%) were suspected of "space-occupying lesions in the pancreatic and biliary system" and 5 (22.73%) were suspected of obstructive jaundice at the outpatient service. Understanding of IgG4-SC was achieved by postoperative pathology in the early stage, then imaging doctors gradually became aware of this disease, and finally multidisciplinary consultation made more physicians understand the disease, which helped to make a confirmed diagnosis earlier. Conclusion For patients with obstructive jaundice, clinical physicians need to identify IgG4-SC based on clinical manifestations and especially imaging features. Multidisciplinary consultation involving imaging and pathology plays an important role in helping clinicians understand this disease and making a confirmed diagnosis.
- Immunoglobulin G,
- Cholangitis, Sclerosing,
- Diagnosis, Differential,
- Therapeutics

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References

[1]	ZEN Y, HARADA K, SASAKI M, et al. IgG4-related sclerosing cholangitis with and without hepatic inflammatory pseudotumor, and sclerosing pancreatitis-associated sclerosing cholangitis: Do they belong to a spectrum of sclerosing pancreatitis?[J]. Am J Surg Pathol, 2004, 28(9): 1193-1203. DOI: 10.1097/01.pas.0000136449.37936.6c.
[2]	HAMANO H, KAWA S, HORIUCHI A, et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis[J]. N Engl J Med, 2001, 344(10): 732-738. DOI: 10.1056/NEJM200103083441005.
[3]	TANAKA A, TAZUMA S, OKAZAKI K, et al. Clinical features, response to treatment, and outcomes of IgG4-related sclerosing cholangitis[J]. Clin Gastroenterol Hepatol, 2017, 15(6): 920-926. e3. DOI: 10.1016/j.cgh.2016.12.038.
[4]	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. DOI: 10.1002/jhbp.596.
[5]	HAN XF, MA X. Immunoglobulin G4-associated cholangitis[J]. Chin J Gastroenteml, 2009, 14(4): 233-236. DOI: 10.3969/j.issn.1008-7125.2009.04.013. 韩小凤, 马雄. IgG4相关性胆管炎[J]. 胃肠病学, 2009, 14(4): 233-236. DOI: 10.3969/j.issn.1008-7125.2009.04.013.
[6]	ZHAO CC, CEN JH, WANG XM et al. Immunoglobulin IgG4 associated cholangitis: A case report[J]. Chin J Surg, 2011, 49(3): 275-276. DOI: 10.3760/cma.j.issn.0529-5815.2011.03.020. 赵超尘, 岑钧华, 王晓明, 等. 免疫球蛋白IgG4相关性胆管炎一例分析[J]. 中华外科杂志, 2011, 49(3): 275-276. DOI: 10.3760/cma.j.issn.0529-5815.2011.03.020.
[7]	FENG YL, YANG AM, YAO F, et al. Clinical features of IgG4-related sclerosing cholangitis: A study of 36 cases[J]. Med J Peking Union Med Coll Hosp, 2015, 6(2): 102-105. DOI: 10.3969/j.issn.1674-9081.2015.02.005. 冯云路, 杨爱明, 姚方, 等. 36例IgG4相关硬化性胆管炎临床特点[J]. 协和医学杂志, 2015, 6(2): 102-105. DOI: 10.3969/j.issn.1674-9081.2015.02.005.
[8]	WANG TL, WU G. Immunoglobulin G4-related sclerosing cholangitis in 34 patients[J]. J Chin Pract Diagn Ther, 2019, 33(9): 887-890. DOI: 10.13507/j.issn.1674-3474.2019.09.014. 王天龙, 吴刚. IgG4相关硬化性胆管炎34例临床分析[J]. 中华实用诊断与治疗杂志, 2019, 33(9): 887-890. DOI: 10.13507/j.issn.1674-3474.2019.09.014.
[9]	XIE QK, SHI XJ, HU YL, et al. IgG4-related sclerosing cholangitis misdiagnosed as hilar cholangiocarcinoma: A case report[J]. J Clin Hepotol, 2018, 34(5): 1094-1095. DOI: 10.3969/j.issn.1001-5256.2018.05.035. 谢乾坤, 石小举, 胡月雷, 等. IgG4相关性硬化性胆管炎误诊为肝门部胆管癌1例报告[J]. 临床肝胆病杂志, 2018, 34(5): 1094-1095. DOI: 10.3969/j.issn.1001-5256.2018.05.035.
[10]	YI Y, SONG BD, LUO RK, et al. IgG4-related sclerosing cholangitis misdiagnosed as hilar cholangiocarcinoma: Case report[J]. Chin J Clinl Med, 2019, 26(1): 154-156. DOI: 10.12025/j.issn.1008-6358.2019.20180165. 易勇, 宋保东, 罗荣奎, 等. IgG4相关性硬化性胆管炎误诊为肝门部胆管癌1例报告[J]. 中国临床医学, 2019, 26(1): 154-156. DOI: 10.12025/j.issn.1008-6358.2019.20180165.
[11]	YI TZ, TANG SH. IgG4-related sclerosing cholangitis misdiagnosed as hilar cholangiocarcinoma: A case report[J]. J Youjiang Med Coll Nationalities, 2017, 39(1): 63-64. DOI: 10.3969/j.issn.1001-5817.2017.01.021. 易廷庄, 汤绍辉. IgG4相关硬化性胆管炎误诊为肝门部胆管癌1例[J]. 右江民族医学院学报, 2017, 39(1): 63-64. DOI: 10.3969/j.issn.1001-5817.2017.01.021.
[12]	ZHOU JB, WANG LM, ZHANG Y, et al. Analysis of misdiagnosis of IgG4 associated cholangitis as hilar cholangiocarcinoma[J]. Clin Misdiagn Misther, 2017, 30(12): 15-17. DOI: 10.3969/j.issn.1002-3429.2017.12.007. 周金宝, 王黎明, 张扬, 等. IgG4相关性胆管炎误诊为肝门部胆管癌分析[J]. 临床误诊误治, 2017, 30(12): 15-17. DOI: 10.3969/j.issn.1002-3429.2017.12.007.
[13]	SUN JY, KONG XY, XIAO CW, et al. IgG4 associated sclerosing cholangitis misdiagnosed as cholangiocarcinoma: Two cases report and literature review[J]. J Hepatopancreatobiliary Surg, 2019, 31(3): 168-171. DOI: 10.11952/j.issn.1007-1954.2019.03.012. 孙江阳, 孔晓宇, 肖朝文, 等. IgG4相关性硬化性胆管炎误诊为胆管癌2例病例分析及文献复习[J]. 肝胆胰外科杂志, 2019, 31(3): 168-171. DOI: 10.11952/j.issn.1007-1954.2019.03.012.
[14]	YAO X, YANG ZH, WANG Z. IgG4 associated cholangitis mimicking hilar cholangiocarcinoma[J]. Chin J Dig, 2013, 33(6): 421-422. DOI: 10.3760/cma.j.issn.0254-1432.2013.06.017. 姚旬, 杨正汉, 王征. 酷似肝门部胆管癌的IgG4相关性胆管炎[J]. 中华消化杂志, 2013, 33(6): 421-422. DOI: 10.3760/cma.j.issn.0254-1432.2013.06.017.
[15]	GONG JL, TAN Z, ZHONG ZD, et al. Two cases of immunoglobulin G4 associated sclerosing cholangitis with suspected hilar cholangiocarcinoma[J]. Chin J Hepatobiliary Surg, 2017, 23(10): 668, 673. DOI: 10.3760/cma.j.issn.1007-8118.2017.10.005. 龚金龙, 谭正, 钟振东, 等. 疑似肝门胆管癌的免疫球蛋白G4相关硬化性胆管炎二例[J]. 中华肝胆外科杂志, 2017, 23(10): 668, 673. DOI: 10.3760/cma.j.issn.1007-8118.2017.10.005.
[16]	ZHANG ZD, XIE WY, LIU YJ, et al. Diagnosis and treatment of simple bile duct involvement with suspected hilar cholangiocarcinoma[J]. Chin J Gen Surg, 2019, 34(1): 82-83. DOI: 10.3760/cma.j.issn.1007-631X.2019.01.026. 张哲栋, 谢文勇, 刘以俊, 等. 疑似肝门部胆管癌的单纯胆管受累IgG4相关性硬化性胆管炎的诊治[J]. 中华普通外科杂志, 2019, 34(1): 82-83. DOI: 10.3760/cma.j.issn.1007-631X.2019.01.026.
[17]	WU H, CHAI LG, PEI F. IgG4-related sclerosing cholangitis: A clinicopathological analysis of two cases[J]. J Diag Pathol, 2016, 23(12): 916-919. DOI: 10.3969/j.issn.1007-8096.2016.12.006. 武慧, 钗丽干, 裴斐. IgG4相关性硬化性胆管炎2例临床病理观察[J]. 诊断病理学杂志, 2016, 23(12): 916-919. DOI: 10.3969/j.issn.1007-8096.2016.12.006.
[18]	LIU W, LIU QF, HONG T, et al. IgG4 associated sclerosing cholangitis suspected of extrahepatic cholangiocarcinoma: A case report[J]. Chin J Hepatobilary Surg, 2015, 21(10): 708-709. DOI: 10.3760/cma.j.issn.1007-8118.2015.10.018. 刘卫, 刘乔飞, 洪涛, 等. 疑似肝外胆管癌的IgG4相关硬化性胆管炎一例[J]. 中华肝胆外科杂志, 2015, 21(10): 708-709. DOI: 10.3760/cma.j.issn.1007-8118.2015.10.018.
[19]	PENG X, QING DX. Delayed diagnosis of IgG4 associated sclerosing cholangitis: A case report[J]. Clin Exp Pathol, 2020, 36(3): 370-371. DOI: 10.13315/j.cnki.cjcep.2020.03.034. 彭欣, 卿笃信. IgG4相关性硬化性胆管炎的延迟诊断1例[J]. 临床与实验病理学杂志, 2020, 36(3): 370-371. DOI: 10.13315/j.cnki.cjcep.2020.03.034.
[20]	ZHANG WW, FAN YG. IgG4-related sclerosing cholangitis: A report of one case[J]. J Clin Hepotol, 2015, 31(9): 1492-1493. DOI: 10.3969/j.issn.1001-5256.2015.09.033. 张温温, 范永刚. IgG4相关性硬化性胆管炎1例报告[J]. 临床肝胆病杂志, 2015, 31(9): 1492-1493. DOI: 10.3969/j.issn.1001-5256.2015.09.033.
[21]	WANG Q, JI YJ, CAI SP, et al. IgG4 associated sclerosing cholangitis misdiagnosed as bile duct tumor: A case report[J]. Chin Hepatol, 2018, 23(2): 189-190. DOI: 10.3969/j.issn.1008-1704.2018.02.029. 王钱, 吉英杰, 蔡少平, 等. IgG4相关硬化性胆管炎误诊为胆管肿瘤1例[J]. 肝脏, 2018, 23(2): 189-190. DOI: 10.3969/j.issn.1008-1704.2018.02.029.
[22]	LIU X, LI JT, FAN YH, et al. A case of IgG4 related disease misdiagnosed as pancreatic cancer[J]. Chin J Rheumatol, 2012, 16(12): 854-855. DOI: 10.3760/cma.j.issn.1007-7480.2012.12.018. 刘霞, 李靖涛, 樊艳华, 等. 误诊为胰腺癌的IgG4相关性疾病一例[J]. 中华风湿病学杂志, 2012, 16(12): 854-855. DOI: 10.3760/cma.j.issn.1007-7480.2012.12.018.
[23]	YANG R, WANG YF. A case of IgG4 related disease misdiagnosed as pancreatic tumor[J]. J Baotou Med Coll, 2017, 33(10): 117. DOI: 10.16833/j.cnki.jbmc.2017.10.051. 杨荣, 王永福. IgG4相关性疾病误诊为胰腺肿瘤1例[J]. 包头医学院学报, 2017, 33(10): 117. DOI: 10.16833/j.cnki.jbmc.2017.10.051.
[24]	BAI L, GUO QL. A case of IgG4 related disease misdiagnosed as pancreatic tumor[J]. Mil Med Sci, 2019, 43(2): 159-160. DOI: 10.7644/j.issn.1674-9960.2019.02.017. 白玲, 郭庆玲. IgG4相关性疾病误诊为胰腺癌1例[J]. 军事医学, 2019, 43(2): 159-160. DOI: 10.7644/j.issn.1674-9960.2019.02.017.
[25]	BI Y, HART PA, LAW R, et al. Obstructive jaundice in autoimmune pancreatitis can be safely treated with corticosteroids alone without biliary stenting[J]. Pancreatology, 2016, 16(3): 391-396. DOI: 10.1016/j.pan.2016.03.017.
[26]	LIU QF, LIU W, HONG T, et al. IgG4-related sclerosing cholangitis: An internal disease calls for the attention of hepatic-biliary-pancreatic surgeons[J]. Med J Peking Union Med Coll Hosp, 2019, 10(3): 201-205. DOI: 10.3969/j.issn.1674-9081.2019.03.003. 刘乔飞, 刘卫, 洪涛, 等. IgG4相关性硬化性胆管炎: 肝胆胰外科医生应该重视的内科疾病[J]. 协和医学杂志, 2019, 10(3): 201-205. DOI: 10.3969/j.issn.1674-9081.2019.03.003.
[27]	SHI YB. CT and MRI diagnosis of IgG4 cholangitis[J]. J Heze Med Coll, 2017, 29(4): 17-19. DOI: 10.3969/j.issn.1008-4118.2017.04.005. 石彦斌. IgG4胆管炎CT诊断以及MRI诊断[J]. 菏泽医学专科学校学报, 2017, 29(4): 17-19. DOI: 10.3969/j.issn.1008-4118.2017.04.005.
[28]	LIU FYY. CT and MRI imaging features and differential diagnosis of IgG4-associated cholangitis[J]. China Modern Med, 2018, 25(3): 76-78. DOI: 10.3969/j.issn.1674-4721.2018.03.025. 刘付燕玉. IgG4相关性胆管炎的CT及MRI影像学特征及鉴别诊断[J]. 中国当代医药, 2018, 25(3): 76-78. DOI: 10.3969/j.issn.1674-4721.2018.03.025.
[29]	LI AQ, WANG Y. CT and MRI imaging features and differential diagnosis of IgG4-associated cholangitis[J]. Chin J Dig Surg, 2015, 14(4): 344-348. DOI: 10.3760/cma.j.issn.1673-9752.2015.04.017. 李安琪, 王屹. IgG4相关性胆管炎CT及MRI影像学诊断与鉴别诊断[J]. 中华消化外科杂志, 2015, 14(4): 344-348. DOI: 10.3760/cma.j.issn.1673-9752.2015.04.017.

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Clinical features, diagnosis, and treatment of IgG4-associated sclerosing cholangitis

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Clinical features, diagnosis, and treatment of IgG4-associated sclerosing cholangitis

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