Presentation and Management of Post-treatment Relapse in Autoimmune Pancreatitis/Immunoglobulin G4-Associated Cholangitis
Presentation and Management of Post-treatment Relapse in Autoimmune Pancreatitis/Immunoglobulin G4-Associated Cholangitis
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DOI：
10.1016/j.cgh.2009.03.021
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发表时间：
2009-10-01
影响因子：
12.6
通讯作者：
Webster, George J. M.
中科院分区：
文献类型：
--
作者：
Sandanayake, Neomal S.;Church, Nicholas I.;Webster, George J. M.
背景与目的：自身免疫性胰腺炎（AIP）是一种多系统疾病，通常有胰腺外表现，如免疫球蛋白G4相关性胆管炎（IAC）。患者对类固醇反应迅速，但治疗后可能复发。我们评估了一组AIP/IAC患者复发的临床管理。方法：我们对2004-2007年诊断为AIP并接受类固醇治疗的患者进行了前瞻性研究。治疗结果在临床、影像学和生化方面被定义为对类固醇的反应、类固醇后的缓解、停用类固醇失败和复发。类固醇+/-硫唑嘌呤（AZA）用于治疗失败、复发或无法戒断类固醇的患者。结果：研究了28例AIP患者; 23例（82%）患有IAC。所有患者在6周内对泼尼松龙治疗有反应。23例患者在中位治疗5个月（范围1.5-17个月）后达到缓解，而S患者（18%）因疾病发作而无法脱机。在达到缓解的患者中，23例患者中有8例（35%）随后复发。总体而言，23例AIP/IAC患者中有13例（57%）复发，而5例孤立AIP患者中无复发（P = 0.04，Fisher精确检验）。所有复发患者均增加/重新开始类固醇治疗; 10例患者还接受了AZA治疗。7例患者达到缓解并维持缓解;他们在中位数14个月（范围，1-27个月）时仍接受AZA单药治疗。结论：46%的AIP患者出现复发或停用类固醇失败。IAC患者复发的风险特别高。AZA似乎对治疗后复发或不能脱离类固醇的患者有效。
BACKGROUND & AIMS: Autoimmune pancreatitis (AIP) is a multisystem disorder that often has extrapancreatic manifestations such as immunoglobulin G4-associated cholangitis (IAC). Patients respond rapidly to steroids but can relapse after therapy. We assessed the clinical management of relapse in a group of patients with AIP/IAC. METHODS: We performed a prospective study of patients diagnosed with AIP from 2004-2007 who received steroids. Treatment outcome was defined clinically, radiologically, and biochemically as response to steroids, remission after steroids, failure to wean steroids, and relapse. Steroids +/- azathioprine (AZA) were used to treat patients who failed, relapsed, or could not be weaned from steroids. RESULTS: Twenty-eight patients with AIP were Studied; 23 (82%) had IAC. All patients responded within 6 weeks to prednisolone therapy. Twenty-three patients achieved remission after a median of 5 months of treatment (range, 1.5-17 months), whereas S patients (18%) could not be weaned because of a disease flare. Of the patients who achieved remission, 8 of 23 (35%) subsequently relapsed. Overall, 13 of 23 patients (57%) with AIP/IAC relapsed, compared with 0 of the 5 with isolated AIP (P = .04, Fisher exact test). Steroids were increased/restarted in all patients who relapsed; 10 also received AZA. Remission was achieved and maintained in 7 patients; they remain on AZA monotherapy at a median of 14 months (range, 1-27 months). CONCLUSIONS: Relapse or failure to wean steroids occurred in 46% of patients with AIP. Patients with IAC are at particularly high risk of relapse. AZA appears to be effective in patients with post-treatment relapse or who cannot be weaned from steroids.