Autoimmune pancreatitis: Clinical and radiological features and objective response to steroid therapy in a UK series
Autoimmune pancreatitis: Clinical and radiological features and objective response to steroid therapy in a UK series
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DOI：
10.1111/j.1572-0241.2007.01531.x
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发表时间：
2007-11-01
影响因子：
9.8
通讯作者：
Webster, George J. M.
中科院分区：
文献类型：
--
作者：
Church, Nicholas I.;Pereira, Stephen P.;Webster, George J. M.
目的：大多数自身免疫性胰腺炎（AIP）病例均来自日本。我们目前的数据在英国的系列，包括临床和放射学特征，在介绍，纵向响应immunosuppress.Methods：在18个月的时间内，所有患者诊断在我们的中心与AIP进行了研究。根据需要进行内镜胆道支架植入术，患者接受泼尼松龙治疗，并纵向评估反应。在类固醇减少后疾病复发的情况下，硫唑嘌呤instituted.Results：11例患者符合AIP的诊断标准。弥漫性胰腺肿大8例（73%），胰管狭窄。7例患者需要胆道支架。所有病例均发生胰腺外受累，包括肝内狭窄和肾脏疾病。开始类固醇治疗8周后，中位血清胆红素水平从38 μ mol/L降至11 μ mol/L（P = 0.001），ALT从97 IU/L降至39 IU/L（P = 0.002）。所有病例均取出支架，无黄疸复发。所有患者的肿块病变和胰胆管狭窄均得到改善。在中位18个月的随访中，6名患者复发，其中4名对硫唑嘌呤有反应。两名患者停止类固醇和保持well.Conclusions：胰腺外疾病是一个重要的特点，AIP在英国系列。免疫抑制治疗的初始反应非常好，但疾病复发很常见。最佳的长期管理仍有待建立。
Objective: Most cases of autoimmune pancreatitis (AIP) have been reported from Japan. We present data on a UK series, including clinical and radiological features at presentation, and longitudinal response to immunosuppression.Methods: Over an 18-month period, all patients diagnosed in our center with AIP were studied. Endoscopic biliary stenting was performed as required, and patients were treated with prednisolone, with response assessed longitudinally. In cases of disease relapse following steroid reduction, azathioprine was instituted.Results: Eleven patients met diagnostic criteria for AIP. Diffuse pancreatic enlargement was seen in eight patients (73%), and pancreatic duct strictures in all. Seven patients required biliary stents. Extrapancreatic involvement occurred in all, including intrahepatic stricturing and renal disease. Eight weeks after starting steroids, the median serum bilirubin level had fallen from 38 mu mol/L to 11 mu mol/L (P = 0.001), and ALT from 97 IU/L to 39 IU/L (P = 0.002). Stents were removed in all cases, with no recurrence of jaundice. Improvements in mass lesions and pancreaticobiliary stricturing occurred in all patients. During a median 18-month follow-up, six patients relapsed, four of whom responded to azathioprine. Two patients discontinued steroids and remained well.Conclusions: Extrapancreatic disease was an important feature of AIP in this UK series. Initial response to immunosuppressive therapy was excellent, but disease relapse was common. Optimal long-term management remains to be established.