Kamisawa Terumi, Kuruma Sawako, Chiba Kazuro
Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan.
Semin Liver Dis. 2016 Aug;36(3):291-6. doi: 10.1055/s-0036-1584324. Epub 2016 Jul 28.
An accurate diagnosis should be made before treatment of autoimmune pancreatitis and immunoglobulin G4-related sclerosing cholangitis. Once a diagnosis has been established, steroids are the standard therapy and symptoms are the major indications. Before steroid therapy, obstructive jaundice and hyperglycemia should be controlled. An initial dose of 0.6 mg/kg/d of oral prednisolone is administered for 2 to 4 weeks, and is gradually tapered over 2 to 3 months. After steroid therapy has begun, blood and imaging tests are performed periodically. Patients with a poor response to steroids should be reevaluated on suspicion of malignancy. To prevent relapse, maintenance therapy using low-dose prednisolone (2.5-5 mg/d) for 1 to 3 years is recommended in Japan. Proximal biliary stricture is reported to be a predictor of relapse. Readministration and dose-up of steroids are effective for relapses. In Western countries, immunosuppressive drugs and rituximab have also been shown to be effective. The optimal treatment regimen should be addressed in future randomized, controlled clinical trials.
在治疗自身免疫性胰腺炎和免疫球蛋白G4相关性硬化性胆管炎之前，应做出准确诊断。一旦确诊，类固醇是标准治疗方法，症状是主要指征。在进行类固醇治疗之前，应控制梗阻性黄疸和高血糖。口服泼尼松龙的初始剂量为0.6mg/kg/d，持续2至4周，然后在2至3个月内逐渐减量。开始类固醇治疗后，应定期进行血液和影像学检查。对类固醇反应不佳的患者若怀疑有恶性肿瘤应重新评估。为防止复发，在日本建议使用低剂量泼尼松龙（2.5 - 5mg/d）进行1至3年的维持治疗。据报道，近端胆管狭窄是复发的一个预测指标。重新使用类固醇并增加剂量对复发有效。在西方国家，免疫抑制药物和利妥昔单抗也已被证明有效。最佳治疗方案应在未来的随机对照临床试验中探讨。
