自身免疫性胰腺炎和IgG4相关硬化性胆管炎的临床诊治  

Clinical Diagnosis and Treatment of Autoimmune Pancreatitis and IgG4-related Sclerosing Cholangitis

作　　者：熊慧芳[1] 刘志坚[1] 舒徐[1] 吕农华[1] 祝荫[1] XIONG Huifang;LIU Zhijian;SHU Xu;LÜ Nonghua;ZHU Yin(Division of Gastroenterology and Hepatology, the First Affiliated Hospital of Nanchang University, Nanchang 330006)

机构地区：[1]南昌大学第一附属医院消化内科,330006

出　　处：《胃肠病学》2021年第7期402-406,共5页Chinese Journal of Gastroenterology

摘　　要：背景:自身免疫性胰腺炎(AIP)是与免疫因素有关的胰腺慢性炎症。IgG4相关硬化性胆管炎(IgG4-SC)是AIP常见的胰腺外表现,其最佳治疗方案需进一步明确。目的:探讨AIP尤其是IgG4-SC的临床诊治方式。方法:南昌大学第一附属医院2015年1月—2021年5月收治的25例AIP患者纳入研究,对其临床、实验室、影像学、病理学表现以及治疗方式和临床结局进行回顾性分析。结果:25例AIP患者均为I型,男女比例为5.25∶1,平均年龄63.5岁。主要临床表现为梗阻性黄疸(56.0%)和上腹痛(44.0%),影像学表现主要为胰腺增大饱满、包鞘征、胆总管下段狭窄继发胆系扩张,伴血清IgG4水平显著升高。14例IgG4-SC患者中,3例总胆红素在1~2倍正常上限(ULN)之间,3例在2~5倍ULN之间,8例>5×ULN。83.3%的患者通过内镜超声引导下细针穿刺抽吸(EUS-FNA)获得病理学诊断。接受单纯激素治疗与接受胆管支架联合或不联合激素治疗的IgG4-SC患者治疗前肝功能无明显差异(P均>0.05),治疗后多数患者肝功能恢复正常。结论:AIP是临床少见疾病,老年男性患者常见,病变常累及胆管引起IgG4-SC,以梗阻性黄疸为首发表现。激素治疗可有效缓解梗阻性黄疸,无需额外的胆管支架置入。Background:Autoimmune pancreatitis(AIP)is a chronic pancreatitis associated with immune factors.IgG4-related sclerosing cholangitis(IgG4-SC)is a common extrapancreatic manifestation of AIP,however,its optimal treatment still needs to be clarified.Aims:To summarize the experience in the clinical diagnosis and treatment of AIP,especially IgG4-SC.Methods:Twenty-five patients with AIP admitted from the First Affiliated Hospital of Nanchang University between January 2015 and May 2021 were recruited,and a retrospective analysis was conducted on the clinical symptoms,laboratory and imaging findings,pathological results,as well as the treatment regimens and clinical outcomes.Results:All the patients were diagnosed as type 1 AIP(84.0%male,mean age 63.5 years).Obstructive jaundice(56.0%)and epigastric pain(44.0%)were the main clinical symptoms.The main imaging findings were diffuse enlargement of the pancreas,capsule-like rim,and distal biliary stricture associated with intra-and extra-hepatic duct dilation.The serum IgG4 elevated significantly.In 14 patients with IgG4-SC,the total bilirubin was between 1-2 upper limit of normal(ULN)in 3 cases,between 2-5 ULN in 3 cases,and>5×ULN in 8 cases.Fifteen(83.3%)patients obtained pathological diagnosis by endoscopic ultrasonography-guided fine-needle aspiration(EUS-FNA).Six IgG4-SC patients were treated with steroids alone,and 6 received biliary stenting with or without steroids,no differences in liver function tests were existed between the two groups prior to treatment(all P>0.05).Liver function tests in most of these patients were normalized after treatment.Conclusions:AIP is a rare disease in clinical practice,and is more frequently seen in elderly male patients.Biliary involvement(IgG4-SC)is common and often presents initially as obstructive jaundice,which can be effectively managed with steroids alone,without the need for biliary stenting.

关 键 词：自身免疫性胰腺炎 黄疸 梗阻性 糖皮质激素类 胆管支架 

分 类 号：R57[医药卫生—消化系统]