Outcome of Patients With Type 1 or 2 Autoimmune Pancreatitis

Outcome of Patients With Type 1 or 2 Autoimmune Pancreatitis
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DOI:
10.1038/ajg.2010.314
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发表时间:
2011-01-01
影响因子:
9.8
通讯作者:
Hammel, Pascal
Hammel, Pascal
中科院分区:
医学1区
文献类型:
--
作者:
Maire, Frederique;Le Baleur, Yann;Hammel, Pascal

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目的:自身免疫性胰腺炎(AIP)的描述比以前更好,但在定义,诊断和治疗方面仍然没有国际共识。我们的目的是分析短期和长期的结果,重点对胰腺内分泌和外分泌功能的患者,寻找复发和胰腺功能不全的预测因素,并比较患者与1型和2型AIP.METHODS:所有连续患者随访AIP在我们的中心1999年和2008年之间。定义了两组:(a)符合HISORt(组织学、影像学、血清学、其他器官受累和对类固醇的反应)标准的1型AIP患者;(B)确诊/可能的2型AIP患者,包括组织学证实的特发性导管中心性胰腺炎(“确诊”)或暗示性影像学、正常血清IgG 4和对类固醇的反应(“可能”)的患者。在随访期间观察AIP相关事件和胰腺外分泌/内分泌功能不全。结果:共纳入44例患者(22例男性),中位年龄37.5(19-73)岁,其中1型AIP患者28例(64%),2型AIP患者16例(36%)。一线治疗包括类固醇激素或胰腺切除术,分别占59和27%的患者。中位随访时间为41(5-130)个月。类固醇在所有治疗的患者中均有效。在中位延迟6个月(1-70)后,在12例患者(27%)中观察到复发。4例患者因类固醇耐药/依赖而接受硫唑嘌呤治疗。高血清IgG 4水平、诊断时疼痛和其他器官受累与复发相关(P
OBJECTIVES: Autoimmune pancreatitis (AIP) is better described than before, but there is still no international consensus for definition, diagnosis, and treatment. Our aims were to analyze the short-and long-term outcome of patients with focus on pancreatic endocrine and exocrine functions, to search for predictive factors of relapse and pancreatic insufficiency, and to compare patients with type 1 and type 2 AIP.METHODS: All consecutive patients followed up for AIP in our center between 1999 and 2008 were included. Two groups were defined: (a) patients with type 1 AIP meeting HISORt (Histology, Imaging, Serology, Other organ involvement, and Response to steroids) criteria; (b) patients with definitive/probable type 2 AIP including those with histologically confirmed idiopathic duct-centric pancreatitis ("definitive") or suggestive imaging, normal serum IgG4, and response to steroids ("probable"). AIP-related events and pancreatic exocrine/endocrine insufficiency were looked for during follow-up. Predictive factors of relapse and pancreatic insufficiency were analyzed.RESULTS: A total of 44 patients (22 males), median age 37.5 (19-73) years, were included: 28 patients (64%) with type 1 AIP and 16 patients (36%) with type 2 AIP. First-line treatment consisted of steroids or pancreatic resection in 59 and 27% of the patients, respectively. Median follow-up was 41 (5-130) months. Steroids were effective in all treated patients. Relapse was observed in 12 patients (27%), after a median delay of 6 months (1-70). Four patients received azathioprine because of steroid resistance/dependence. High serum IgG4 level, pain at time of diagnosis, and other organ involvement were associated with relapse (P