【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。更多还原

【Abstract】 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.更多还原