Advances in diagnostic difficulties and targeted therapy of IgG4-related sclerosing cholangitis
10.3760/cma.j.cn115610-20250501-00175
- VernacularTitle:IgG4相关硬化性胆管炎的诊断难点与靶向治疗进展
- Author:
Xingkun LIAO
1
;
Zhiwei HE
1
;
Yihua WANG
1
;
Futang LI
1
;
Chao YU
1
Author Information
1. 贵州医科大学附属医院肝胆外科，贵阳 550001
- Publication Type:Journal Article
- Keywords:
IgG4-related sclerosing cholangitis;
Immune-mediated diseases;
Clinical hetero-geneity and misdiagnosis;
Glucocorticosteroid treatment;
Targeted therapy
- From:
Chinese Journal of Digestive Surgery
2025;24(7):921-926
- CountryChina
- Language:Chinese
-
Abstract:
IgG4-related sclerosing cholangitis (IgG4-SC) is an IgG4-related disease charac-terized by bile duct wall thickening and luminal stricture. Its pathogenesis involves complex multi-dimensional regulation, including immune imbalance, genetic epigenetic regulation, microbial ecolo-gical abnormalities, and activation of fibrotic pathways. Due to the lack of specificity in clinical mani-festations, IgG4-SC is easily misdiagnosed as biliary tract diseases such as cholangiocarcinoma and primary sclerosing cholangitis in the early stage, leading to diagnostic difficulties. At present, gluco-corticoids are the preferred treatment option. Although most patients can quickly recover in the early stages, high recurrence rates and hormone dependence limit the long-term effectiveness of treatment. The authors combine the latest research progress at home and abroad to deeply analyze the pathogenesis, clinical manifestations, and diagnosis of IgG4-SC, focusing on treatment strategies and cutting-edge advances, in order to provide reference and inspiration for clinical practice.
