Pan X X, Wu Z W
State Key Laboratory for Diagnosis and Treatment of Infectious Diseases, National Clinical Research Centre for Infectious Diseases, Collaborative Innovation Centre for Diagnosis and Treatment of Infectious Diseases, the First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou 310000, China.
Zhonghua Gan Zang Bing Za Zhi. 2022 Apr 20;30(4):452-456. doi: 10.3760/cma.j.cn501113-20210907-00458.
IgG4-related disease (IgG4-RD) is an immune-mediated condition associated with chronic fibroinflammatory lesions that can affect nearly any organ. IgG4-related hepatobiliary and pancreatic diseases are IgG4-RD involving the hepatobiliary and pancreatic system, which is characterized with elevated serum IgG4 concentrations, large numbers of IgG4 positive lymphoplasma cells infiltration in affected organs, storiform fibrosis, and imaging changes of organ morphology. Due to the lack of reliable biomarkers, histopathology is still an important basis for diagnosis. The pathogenesis of IgG4-related hepatobiliary and pancreatic diseases has not been clarified. This review focuses on the recent advances in intestinal microecology-immunology, host genetics-immunity and recurrence monitoring of IgG4-related hepatobiliary and pancreatic diseases.
IgG4相关疾病（IgG4-RD）是一种与慢性纤维炎症性病变相关的免疫介导疾病，几乎可累及任何器官。IgG4相关肝胆胰疾病是累及肝胆胰系统的IgG4-RD，其特征为血清IgG4浓度升高、受累器官中有大量IgG4阳性淋巴浆细胞浸润、席纹状纤维化以及器官形态的影像学改变。由于缺乏可靠的生物标志物，组织病理学仍是诊断的重要依据。IgG4相关肝胆胰疾病的发病机制尚未阐明。本文综述了IgG4相关肝胆胰疾病在肠道微生态-免疫学、宿主遗传学-免疫以及复发监测方面的最新进展。
