Abstract

The effectiveness of azathioprine (AZA) in preventing relapse and maintaining autoimmune pancreatitis (AIP) remission has been reported; however, most of these studies are case series with no randomized control trials available in the literature. Therefore, this study performed a systematic review and meta-analysis of the existing literature on this subject to determine the clinical efficacy of AZA as maintenance therapy for AIP patients. A systematic search was performed to identify studies on the clinical efficacy of AZA as maintenance therapy in AIP patients. The crude multiple relapse rate was estimated to assess the ability of AZA to control relapses in AIP. Pooled estimates were obtained using a random-effects model with the DerSimonian-Laird method. We identified AIP patients who did not respond to initial steroid treatment, experienced steroid weaning failure, or those who relapsed during remission as refractory cases. After reviewing the studies, ten articles fulfilled the inclusion criteria and were selected for meta-analysis. Of all 4504 patients, 3534 patients were treated with steroids, and 346 patients were treated with AZA for relapsed AIP. In this meta-analysis, 14/73 (19.2%) patients receiving AZA for refractory AIP relapsed. Meanwhile, 14/47 (29.8%) patients without AZA experienced relapse. The integrated odds ratio for relapse risk in patients receiving AZA was estimated to be 0.52 (p = 0.15). This systematic review and meta-analysis demonstrated the efficacy of AZA in preventing relapse of AIP, which supports the use of AZA as a maintenance treatment in patients with AIP who relapse upon withdrawal of steroid therapy.

Highlights

  • With progress in research on autoimmune pancreatitis (AIP), the number of AIP patients in Japan has increased [1]

  • We identified AIP patients who did not respond to initial steroid treatment, experienced steroid weaning failure, or those who relapsed during remission as refractory cases

  • This systematic review and meta-analysis of collected data from 10 studies suggested the efficacy of AZA in preventing AIP relapse, which supports AZA as a maintenance

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Summary

Introduction

With progress in research on autoimmune pancreatitis (AIP), the number of AIP patients in Japan has increased [1]. Regarding a clinical study on the prevention of AIP relapse, Kamisawa et al examined the efficacy of low-dose daily prednisone (PSL) (2.5–10 mg) in AIP patients and reported that the relapse rates in the steroid continued group and the discontinued group were 23% and 34%, respectively (p = 0.048) [3]. This study demonstrated that maintenance therapy with CS at 5–7.5 mg/day for three years might decrease relapses in AIP patients compared to those who discontinued the treatment at 26 weeks. Based on these findings, the continuation of low-dose CS may be useful for maintaining AIP remission

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