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-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.
作者
王天琪
刘燕鹰
WANG Tianqi;LIU Yanying(Department of Rheumatology and Immunology,Beijing Friendship Hospital,Captital Medical University,Beijing 100050,China)
出处
《临床肝胆病杂志》
CAS
北大核心
2022年第4期762-766,共5页
Journal of Clinical Hepatology
基金
首都卫生发展科研专项(首发2022-2-2026)。
关键词
IgG4相关疾病
胰腺炎
胆管炎
硬化性
肝疾病
IgG4-Related Diseases
Pancreatitis
Cholangitis
Sclerosing
Liver Diseases
