病例报告：孤立性免疫球蛋白G4相关性硬化性胆管炎误诊为肝门部胆管癌。
Case report: Isolated immunoglobulin G4-related sclerosing cholangitis misdiagnosed as hilar cholangiocarcinoma.
作者信息
Li Hui, Wang Ran, Wang Dongyang, Tang Yufu, Liu Xuantong, Li Hongyu, Qi Xingshun
机构信息
Department of Gastroenterology, General Hospital of Northern Theater Command, Shenyang, China.
Department of Life Sciences and Biopharmaceutics, Shenyang Pharmaceutical University, Shenyang, China.
出版信息
Front Oncol. 2024 May 23;14:1385214. doi: 10.3389/fonc.2024.1385214. eCollection 2024.
BACKGROUND
Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is frequently accompanied with type 1 autoimmune pancreatitis (AIP). Isolated IgG4-SC which is not accompanied with AIP is uncommon in clinical practice, and its manifestations are similar to those of hilar cholangiocarcinoma.
CASE PRESENTATION
A 55-year-old male presented with persistent aggravation of icteric sclera and skin. He was initially diagnosed with hilar cholangiocarcinoma and underwent surgery. However, positive IgG4 plasma cells were found in the surgical specimens. Thus, a pathological diagnosis of IgG4-SC was established. After that, steroid therapy was given and initially effective. But he was steroid dependent, and then received rituximab therapy twice. Unfortunately, the response to rituximab therapy was poor.
CONCLUSION
It is crucial to differentiate isolated IgG4-SC from hilar cholangiocarcinoma to avoid unnecessary surgery. Future studies should further explore effective treatment strategy in patients who do not respond to steroids therapy. It is also required to develop novel and accurate diagnostic approaches to avoid unnecessary surgical procedures.
背景
免疫球蛋白G4相关性硬化性胆管炎（IgG4-SC）常伴有1型自身免疫性胰腺炎（AIP）。临床实践中，不伴有AIP的孤立性IgG4-SC并不常见，其表现与肝门部胆管癌相似。
病例介绍
一名55岁男性，巩膜及皮肤黄疸持续加重。他最初被诊断为肝门部胆管癌并接受了手术。然而，手术标本中发现了阳性IgG4浆细胞。因此，确诊为IgG4-SC。此后，给予类固醇治疗，最初有效。但他对类固醇产生依赖，随后接受了两次利妥昔单抗治疗。不幸的是，利妥昔单抗治疗效果不佳。
结论
区分孤立性IgG4-SC与肝门部胆管癌以避免不必要的手术至关重要。未来的研究应进一步探索对类固醇治疗无反应患者的有效治疗策略。还需要开发新的、准确的诊断方法以避免不必要的手术操作。
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