目的： 总结1型自身免疫性胰腺炎（autoimmune pancreatitis, AIP）的临床特点及诊治经验。方法： 本研究对2012年10月至2018年10月复旦大学附属中山医院收治的1型AIP病人9例,回顾性分析临床表现和实验室、影像学、组织病理学检查以及治疗、预后。结果： 9例1型AIP病例临床表现为黄疸、腹胀、腹痛、体重减轻和食欲下降。胰腺外受累器官为胆管、淋巴结、肾脏和腹膜后组织。9例IgG4均升高,8例>2倍正常值上限。上腹部MRI检查以全胰肿大为主（7例）,2例提示胰腺内占位,3例见胰周包膜样边缘。2例胰腺细针穿刺组织学检查未得出诊断。所有病人均行类固醇治疗,疗效较好。治疗初期内镜逆行胰胆管造影放置胆道塑料支架,分别出现肝脓肿和胆总管结石各1例。复发病人使用激素或激素联合免疫抑制剂仍获缓解。结论： 1型AIP仅部分病人有典型影像学表现,需慎重诊断并系统评估,激素治疗有效。
Objective To summarize the clinical characteristics, diagnosis and therapy of type 1 autoimmune pancreatitis (AIP). Methods Totally 9 patients diagnosed with type 1 AIP in Zhongshan Hospital from October 2012 to October 2018 were enrolled. Retrospective analysis of clinical manifestations, laboratory tests, imaging, histopathological examination, treatment and prognosis was conducted. Results Patients commonly presented jaundice, bloating, abdominal ache, both appetite and weight loss. The most common other organ involvements (OOI) were bile duct, lymph nodes, kidney and retroperitoneal tissue in order. All 9 cases had increased serum immunoglobulin G4 levels and 8 cases had 2 times higher than upper limits of normal levels. Upper abdominal MRI showed pancreatic enlargement in 7 cases, pancreatic mass in 2 cases and envelope-like edges in 3 cases. No histopathological diagnosis was obtained with tissues from fine needle aspiration in 2 cases. All the patients received steroid treatment with well results. Two cases who underwent endoscopic retrograde biliary drainage were found liver abscesses and common bile duct stone respectively. Recurrent cases still were improved after steroid retreatment or steroid combined with immune inhibitor usage. Conclusions Only less patients with type 1 AIP have typical imaging findings. Type 1 AIP needs to be diagnosed carefully and evaluated systematically. Treatment of type 1 AIP with steroid could be effective.
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