免疫球蛋白G4相关肝胆胰疾病研究进展与展望

Progress and prospects of immunoglobulin G4-related hepatobiliary-pancreatic disease

免疫球蛋白G4(immunoglobulin G4,IgG4)相关肝胆胰疾病是以血清IgG4水平升高、胆管或腺管周围大量IgG4阳性浆细胞浸润为特征,同时可累及几乎全身所有器官的自身免疫性疾病。对疑似IgG4相关肝胆胰疾病的患者需结合组织学、血清IgG4水平、受累器官影像、临床表现等作出诊断。尽管其作为一类新近认识的自身免疫性疾病受到学者的广泛关注,但发病机制仍未完全明确,目前研究主要集中于遗传、天然和适应性免疫、自身抗原及IgG4等方面。本文对IgG4相关肝胆胰疾病领域近5年来取得的临床和基础研究进展以及尚存的难点和挑战进行综述。

Immunoglobulin G4(IgG4)-related hepatobiliary-pancreatic diseases is characterized with elevated serum immunoglobulin G4 levels,large numbers of IgG4 positive plasma cells infiltration around biliary and pancreatic ducts,it is categorized as autoimmune liver disease which can affect almost any organ.Diagnosis of IgG4-related hepatobiliary-pancreatic diseases relies on combination of histology,serum IgG4 levels,imaging and clinical presentation.Although recognized as a novel autoimmune disease,the pathogenesis remains uncertain,current studies are focus on genetic,innate and adaptive immunity,autoantigen and IgG4.This review will update recent 5 years findings on the basic and clinical research and propose remaining difficulties and challenge in IgG4-related hepatobiliary-pancreatic diseases.