Division of Digestive Diseases, Department of Medicine, Emory University School of Medicine, Atlanta, GA 30322, USA.
World J Gastroenterol. 2012 May 14;18(18):2287-90. doi: 10.3748/wjg.v18.i18.2287.
Systemic corticosteroids represent the standard treatment for autoimmune pancreatitis with IgG4-associated cholangitis. For steroid-dependent disease, azathioprine has been used for maintenance of remission. Mycophenolate mofetil has been used for transplant immunosuppression and more recently for autoimmune hepatitis; however, there are no case reports to date on the use of mycophenolate mofetil in adult patients with autoimmune pancreatitis. A patient with IgG4-mediated autoimmune pancreatitis and IgG4-associated cholangitis refractory to steroids and intolerant of azathioprine was treated with mycophenolate mofetil, which inhibits de novo guanosine synthesis and blockade of both B and T lymphocyte production. Introduction of mycophenolate mofetil and uptitration to 1000 mg by mouth twice daily over a treatment period of 4 mo was associated with improvement in the patient's energy level and blood glucose control and was not associated with any adverse events. The patient was managed without a biliary stent. However, there was a return of symptoms, jaundice, increase in transaminases, and hyperbilirubinemia when the prednisone dose reached 11 mg per day. In the first report of mycophenolate mofetil use in an adult patient with IgG4-associated autoimmune pancreatitis and IgG4-associated cholangitis, the introduction of mycophenolate mofetil was safe and well-tolerated without adverse events, but it did not enable discontinuation of the steroids. Mycophenolate mofetil and other immunomodulatory therapies should continue to be studied for maintenance of remission in the large subset of patients with refractory or recurrent autoimmune pancreatitis.
系统性皮质类固醇是 IgG4 相关胆管炎自身免疫性胰腺炎的标准治疗方法。对于依赖于类固醇的疾病,硫唑嘌呤已被用于维持缓解。霉酚酸酯已被用于移植免疫抑制,最近也用于自身免疫性肝炎;然而,迄今为止,尚无关于霉酚酸酯在 IgG4 介导的自身免疫性胰腺炎成年患者中的使用的病例报告。一名 IgG4 介导的自身免疫性胰腺炎和 IgG4 相关胆管炎患者对类固醇耐药且不耐受硫唑嘌呤,用霉酚酸酯治疗,霉酚酸酯抑制从头鸟苷酸合成并阻断 B 和 T 淋巴细胞产生。霉酚酸酯的引入和滴定至每天口服 1000mg,分两次,治疗 4 个月与患者的能量水平和血糖控制改善相关,且无任何不良事件。患者未放置胆管支架进行管理。然而,当泼尼松剂量达到每天 11mg 时,出现了症状、黄疸、转氨酶升高和高胆红素血症的复发。在首例 IgG4 相关自身免疫性胰腺炎和 IgG4 相关胆管炎成人患者使用霉酚酸酯的报告中,霉酚酸酯的引入是安全且耐受良好的,没有不良事件,但它并不能停用类固醇。霉酚酸酯和其他免疫调节疗法应继续用于研究,以维持大部分难治性或复发性自身免疫性胰腺炎患者的缓解。