IgG4相关肝胆胰疾病诊疗进展

王天琪; 刘燕鹰

doi:10.3969/j.issn.1001-5256.2022.04.006

IgG4相关肝胆胰疾病诊疗进展

DOI: 10.3969/j.issn.1001-5256.2022.04.006

王天琪,
刘燕鹰^, ,

首都医科大学附属北京友谊医院风湿内科，北京 100050

基金项目:

首都卫生发展科研专项 (2022-2-2026)

利益冲突声明：所有作者均声明不存在利益冲突。

作者贡献声明：王天琪负责文献检索、撰写文章；刘燕鹰负责文章的整体指导、审核及修改完善。

详细信息

通信作者:
刘燕鹰，57495011@qq.com

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出版历程
- 收稿日期: 2022-01-11
- 录用日期: 2022-02-13
- 出版日期: 2022-04-20

Advances in diagnosis and treatment of IgG4-related hepatobiliary and pancreatic diseases

Tianqi WANG,
Yanying LIU^{,
,}

Department of Rheumatology and Immunology, Beijing Friendship Hospital, Captital Medical University, Beijing 100050, China

Research funding:

Capital's Fund for Healthy Improvement and Researth (2022-2-2026)

More Information

Corresponding author: LIU Yanying, 57495011@qq.com(ORCID: 0000-0003-2609-4052)

摘要

摘要: IgG4相关肝胆胰疾病是IgG4相关疾病多器官纤维化炎性疾病的一部分，包括IgG4相关自身免疫性胰腺炎、IgG4相关硬化性胆管炎、IgG4相关肝脏病变。主要病理表现为IgG4⁺浆细胞、淋巴细胞浸润，伴有席纹状纤维化、闭塞性静脉炎和嗜酸性粒细胞浸润。临床诊断多采用IgG4相关疾病综合诊断标准和专科制定的特异性器官受累诊断标准，难点在于与肿瘤性疾病的鉴别，新型诊断标志物有望提高诊断敏感度及特异度。迄今，一线治疗药物仍为糖皮质激素，生物制剂，尤其是CD20单抗疗效肯定，对于激素禁忌/不耐受或复发难治性患者不失为一种选择。
- IgG4相关疾病 /
- 胰腺炎 /
- 胆管炎, 硬化性 /
- 肝疾病
Abstract: IgG4-related hepatobiliary and pancreatic diseases are a part of the IgG4-related disease multiorgan fibroinflammatory disorder, including IgG4-related autoimmune pancreatitis, IgG4-related sclerosing cholangitis, and IgG4-related hepatic involvement. The main pathological features include IgG4⁺ plasma cell/lymphocyte infiltration, storiform fibrosis, obliterative phlebitis, and eosinophil infiltration. The diagnosis of this disease is often based on the comprehensive diagnostic criteria for IgG4-related diseases and organ-specific diagnostic criteria. However, it is difficult to differentiate IgG4-related hepatobiliary and pancreatic diseases from neoplastic diseases, and novel diagnostic biomarkers are expected to improve the sensitivity and specificity of diagnosis. To date, glucocorticoids remain the first-line drug for this disease, and biological agents, especially anti-CD20 monoclonal antibody, may be an alternative therapy for patients with corticosteroid contraindication/intolerance or recurrent/refractory disease.
- IgG4-Related Diseases /
- Pancreatitis /
- Cholangitis, Sclerosing /
- Liver Diseases

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参考文献(46)

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