IgG4相关性硬化性胆管炎误诊为胆管癌的1例病例报告
A Case Report of IgG4-Related Sclerosing Cholangitis Misdiagnosed as Cholangiocarcinoma
DOI: 10.12677/hjbm.2026.164079, PDF,   
作者: 吴 浪*：大理大学临床医学院，云南 大理；程 伟, 赵鹏举, 施海斌, 黎 川, 石洪波：大理大学第一附属医院胃肠外科，云南 大理；杨继武#：大理大学临床医学院，云南 大理；大理大学第一附属医院胃肠外科，云南 大理
关键词: IgG4相关性硬化性胆管炎；IgG4相关性疾病；误诊；I型自身免疫性胰腺炎；胆管癌；IgG4-Related Sclerosing Cholangitis； IgG4-Related Disease； Misdiagnosis； Type I Autoimmune Pancreatitis； Cholangiocarcinoma
摘要: 目的：探讨IgG4相关性硬化性胆管炎(IgG4-SC)的临床表现、误诊误治原因及防范措施。方法：对2025年6月误诊为胆管癌(CCA)的一例IgG4-SC患者的临床资料、实验室检查、影像学检查和活组织病理检查进行回顾性分析。结果：本例因突发黄疸入院，行实验室检查、影像学检查等，初步诊断为肝门部胆管癌(HCCA)。拟给予患者手术治疗，但因发现患者血清IgG水平异常，借阅外院活组织病理切片重新检查，最终确诊为伴I型自身免疫性胰腺炎(AIP)的IgG4-SC。停止手术后给予患者糖皮质激素治疗，患者病情好转。3周后复查腹部计算机断层扫描(CT)显示胆道未见狭窄。结论：IgG4-SC为临床上较少见的胆道狭窄性疾病，临床表现及影像学特征与胆管癌高度相似，给其诊断带来巨大挑战，对于可疑的胆管病变，在鉴别诊断中应该充分考虑IgG4-SC，结合患者血清学IgG4检查及活组织病理检查可有助于IgG4-SC的诊断和治疗。
Abstract: Objective: This paper aims to explore the clinical manifestations, causes of misdiagnosis and mismanagement, and preventive measures of IgG4-related sclerosing cholangitis (IgG4-SC). Methods: A retrospective analysis was conducted on the clinical data, laboratory tests, imaging examinations, and histopathological examinations of a patient with IgG4-SC who was misdiagnosed as cholangiocarcinoma (CCA) in June 2025. Results: This patient was admitted due to sudden jaundice and underwent laboratory tests, imaging examinations, etc. The initial diagnosis was hilar cholangiocarcinoma (HCCA). The patient was planned for surgical treatment, but due to the abnormal serum IgG level found, the biopsy from another hospital was borrowed for re-examination, and the patient was finally diagnosed as IgG4-SC with type I autoimmune pancreatitis (AIP). After stopping the surgery, the patient was treated with glucocorticoids, and the condition improved. Three weeks later, a re-examination of abdominal computed tomography (CT) showed no stenosis in the bile duct. Conclusion: IgG4-SC is a relatively rare biliary stenosis disease in clinical practice. Its clinical manifestations and imaging features are highly similar to cholangiocarcinoma, posing great challenges for its diagnosis. For suspected biliary lesions, IgG4-SC should be fully considered in differential diagnosis. Combining the patient’s serological IgG4 test and histopathological examination can help with the diagnosis and treatment of IgG4-SC.
文章引用：吴浪, 程伟, 赵鹏举, 施海斌, 黎川, 石洪波, 杨继武. IgG4相关性硬化性胆管炎误诊为胆管癌的1例病例报告[J]. 生物医学, 2026, 16(4): 776-783. https://doi.org/10.12677/hjbm.2026.164079

参考文献

[1] Drazilova, S., Veseliny, E., Lenartova, P.D., Drazilova, D., Gazda, J., Grgurevic, I., et al. (2021) IgG4‐Related Sclerosing Cholangitis: Rarely Diagnosed, but Not a Rare Disease. Canadian Journal of Gastroenterology and Hepatology, 2021, Article ID: 1959832.
https://doi.org/10.1155/2021/1959832
[2] Tanaka, A., Mori, M., Kubota, K., Naitoh, I., Nakazawa, T., Takikawa, H., et al. (2020) Epidemiological Features of Immunoglobulin G4‐Related Sclerosing Cholangitis in Japan. Journal of Hepato-Biliary-Pancreatic Sciences, 27, 598-603.
https://doi.org/10.1002/jhbp.793
[3] Li, H., Wang, R., Wang, D., Tang, Y., Liu, X., Li, H., et al. (2024) Case Report: Isolated Immunoglobulin G4-Related Sclerosing Cholangitis Misdiagnosed as Hilar Cholangiocarcinoma. Frontiers in Oncology, 14, Article ID: 1385214.
https://doi.org/10.3389/fonc.2024.1385214
[4] Tanaka, A., Tazuma, S., Okazaki, K., Nakazawa, T., Inui, K., Chiba, T., et al. (2017) Clinical Features, Response to Treatment, and Outcomes of IgG4-Related Sclerosing Cholangitis. Clinical Gastroenterology and Hepatology, 15, 920-926.E3.
https://doi.org/10.1016/j.cgh.2016.12.038
[5] Hori, Y., Chari, S.T., Tsuji, Y., Takahashi, N., Inoue, D., Hart, P.A., et al. (2021) Diagnosing Biliary Strictures: Distinguishing IgG4-Related Sclerosing Cholangitis from Cholangiocarcinoma and Primary Sclerosing Cholangitis. Mayo Clinic Proceedings: Innovations, Quality & Outcomes, 5, 535-541.
https://doi.org/10.1016/j.mayocpiqo.2021.03.005
[6] Smit, W.L., Culver, E.L. and Chapman, R.W. (2016) New Thoughts on Immunoglobulin G4-Related Sclerosing Cholangitis. Clinics in Liver Disease, 20, 47-65.
https://doi.org/10.1016/j.cld.2015.08.004
[7] Sah, R.P., Pannala, R., Chari, S.T., Sugumar, A., Clain, J.E., Levy, M.J., et al. (2010) Prevalence, Diagnosis, and Profile of Autoimmune Pancreatitis Presenting with Features of Acute or Chronic Pancreatitis. Clinical Gastroenterology and Hepatology, 8, 91-96.
https://doi.org/10.1016/j.cgh.2009.09.024
[8] Björnsson, E., Chari, S.T., Smyrk, T.C. and Lindor, K. (2007) Immunoglobulin G4 Associated Cholangitis: Description of an Emerging Clinical Entity Based on Review of the Literature. Hepatology, 45, 1547-1554.
https://doi.org/10.1002/hep.21685
[9] Dumrisilp, T. and Tuchinda, N. (2025) Atypical Presentation of Immunoglobulin G4-Related Sclerosing Cholangitis with a Coexistent Gallbladder Hydrops in a Child. Pediatrics & Neonatology, 66, 396-397.
https://doi.org/10.1016/j.pedneo.2025.01.009
[10] Sabbahia, D., Msaaf, H., Atrassi, M., Moukhlis, S., Bennani, N. and Abkari, A. (2024) IgG4 Sclerosing Cholangitis: Entity Rarely Described in Children. American Journal of Pediatrics, 10, 158-161.
https://doi.org/10.11648/j.ajp.20241003.19
[11] Miki, A., Sakuma, Y., Ohzawa, H., Sanada, Y., Sasanuma, H., Lefor, A.T., et al. (2015) Immunoglobulin G4-Related Sclerosing Cholangitis Mimicking Hilar Cholangiocarcinoma Diagnosed with Following Bile Duct Resection: Report of a Case. International Surgery, 100, 480-485.
https://doi.org/10.9738/intsurg-d-14-00230.1
[12] Okut, G., Ugur, M.A., Yildiz, H., et al. (2025) Isolated IgG4-Related Sclerosing Cholangitis Mimicking Hilar Cholangiocarcinoma: A Case Report and Review. Hepatology Forum, 6, 166-169.
https://doi.org/10.14744/hf.2025.2025.0003
[13] Ohara, H., Okazaki, K., Tsubouchi, H., Inui, K., Kawa, S., Kamisawa, T., et al. (2012) Clinical Diagnostic Criteria of IgG4‐Related Sclerosing Cholangitis 2012. Journal of Hepato-Biliary-Pancreatic Sciences, 19, 536-542.
https://doi.org/10.1007/s00534-012-0521-y
[14] Ghazale, A., Chari, S.T., Zhang, L., Smyrk, T.C., Takahashi, N., Levy, M.J., et al. (2008) Immunoglobulin G4-Associated Cholangitis: Clinical Profile and Response to Therapy. Gastroenterology, 134, 706-715.
https://doi.org/10.1053/j.gastro.2007.12.009
[15] Nakazawa, T., Ohara, H., Sano, H., Aoki, S., Kobayashi, S., Okamoto, T., et al. (2004) Cholangiography Can Discriminate Sclerosing Cholangitis with Autoimmune Pancreatitis from Primary Sclerosing Cholangitis. Gastrointestinal Endoscopy, 60, 937-944.
https://doi.org/10.1016/s0016-5107(04)02229-1
[16] Naitoh, I., Yoshida, M. and Nakazawa, T. (2025) Endoscopic Diagnosis of Immunoglobulin G4‐Related Sclerosing Cholangitis. Digestive Endoscopy, 37, 824-833.
https://doi.org/10.1111/den.15039
[17] Nakazawa, T., Naitoh, I., Hayashi, K., Okumura, F., Miyabe, K., Yoshida, M., et al. (2012) Diagnostic Criteria for IgG4-Related Sclerosing Cholangitis Based on Cholangiographic Classification. Journal of Gastroenterology, 47, 79-87.
https://doi.org/10.1007/s00535-011-0465-z
[18] Kamisawa, T., Nakazawa, T., Tazuma, S., Zen, Y., Tanaka, A., Ohara, H., et al. (2019) Clinical Practice Guidelines for IgG4‐Related Sclerosing Cholangitis. Journal of Hepato-Biliary-Pancreatic Sciences, 26, 9-42.
https://doi.org/10.1002/jhbp.596
[19] Bochatay, L., Majno, P., Giostra, E. and Frossard, J.L. (2016) Isolated Liver Hilar Infiltration by IgG4 Inflammation Mimicking Cholangiocarcinoma. Case Reports in Gastroenterology, 10, 512-517.
https://doi.org/10.1159/000448989
[20] Stone, J.H., Khosroshahi, A., Zhang, W., Della Torre, E., Okazaki, K., Tanaka, Y., et al. (2025) Inebilizumab for Treatment of IgG4-Related Disease. New England Journal of Medicine, 392, 1168-1177.
https://doi.org/10.1056/nejmoa2409712

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