Elsevier

Pancreatology

Volume 17, Issue 1, January–February 2017, Pages 1-6
Pancreatology

International consensus for the treatment of autoimmune pancreatitis

https://doi.org/10.1016/j.pan.2016.12.003Get rights and content

Abstract

Background and aims

The International Consensus Diagnostic Criteria (ICDC) for AIP has proposed two distinctive type of AIP, type 1 and type 2, and enabled us first to differentiate two types of AIP each other. By initial steroid treatment for induction of remission, remission can be successfully induced in almost all subjects with type 1 and type 2 AIP. As relapse rate in type 1 AIP is significantly higher than in type 2 AIP, there has been ongoing debate on how to treat effectively relapse of type 1 AIP.

Methods

By a modified Delphi approach, a panel of international experts has proposed an international consensus on the treatment of AIP after intense discussion and deliberation during an international consensus symposium of the International Association of Pancreatology (IAP) 2016.

Results

Individual statements for nine clinical questions with recommendation levels and the therapeutic strategy have been proposed.

Conclusion

The recommendations are based on the available evidence, and eastern and western experts' opinions to find standard treatment of AIP worldwide. These recommendations can be tailored according to the local expertise and context in the management of individual patients.

Introduction

Autoimmune pancreatitis (AIP) is a distinct form of pancreatitis characterized clinically by frequent presentation with obstructive jaundice with or without a pancreatic mass, histologically by a lymphoplasmacytic infiltrate and fibrosis, and therapeutically by a dramatic response to steroids [1]. The International Consensus Diagnostic Criteria (ICDC) for AIP has proposed two distinctive type of AIP, type 1 and type 2, and enabled us first to differentiate two types of AIP from each other [1]. Recently, type 1 AIP has been defined as a pancreatic manifestation of IgG4-related disease [2], [3].

In an international multicenter retrospective study using more than one thousand cases, the majority (74%) of subjects with type 1 AIP were initially treated with steroids, rather than surgical or conservative treatments, in comparison with type 2 subjects in which only 62% were treated with steroids (p = 0.01) [4]. By initial steroid treatment for induction of remission, remission has been successfully induced in almost all subjects with type 1 and type 2 AIP. The patients with type 1 who received intervention (either steroids or surgery) showed higher remission rates (90–99.2%) compared with those who did not receive it (55.2–74%) [4], [5]. On the other hand, initial remission rates were similar in patients with type 2 AIP who received intervention compared with conservative management (83.5% vs 66.7%, respectively, p = 0.29) [4]. Relapse rate in type 1 AIP (31%) is significantly higher than in type 2 AIP (9%) [4], [5], [6], [7], [8]. Due to the high relapse in type 1 AIP, the concepts of induction of remission and maintenance of remission have been proposed.

There has been ongoing debate on how to treat the patients with AIP. Questions that need consensus include: Who should be treated? In patients with obstructive jaundice who needs biliary drainage before treatment of AIP? What is the induction regimen for steroid treatment? When to taper and when to stop initial steroid treatment? Who needs and who does not need maintenance treatment? How to treat relapsing patients?

To achieve the international consensus for treatment of AIP, the international consensus symposium was organized in the 20th meeting of the International Association of Pancreatology (IAP) on the 4th∼7th July 2016 held in Sendai, Japan. The moderators selected eastern and western expert panels based on previous international meetings and discussions through email or physical meetings for AIP. Prior to the consensus meeting, the panels extracted the specific clinical questions and statements for treatment of AIP from the literature by PubMed search (1963–2016). Most of the evidence levels of the specific clinical statements and a secondary database were still lower than grade III as proposed by the Agency for Health Care Policy and Research in 1993. Therefore, we have developed the consensus guidelines for treatment of AIP by modified Delphi approach. To establish consensus, a panel of international experts has proposed 9 clinical questions and statements on the treatment of AIP after intense discussion and deliberation during the symposium. The recommendations are based on the available evidence, and eastern and western experts' opinions to find standard treatment of AIP worldwide. Because available clinical evidence regarding the management of AIP is limited, we could not set a suitable recommendation level for some clinical statements according to an evidence based system such as GRADE system. We evaluated some as ‘‘strongly recommendable’’, (level A) or ‘‘strongly unrecommendable (level D)’’, and ‘‘ordinarily recommendable’ ’(level B), ‘‘unrecommendable’’ (level C), or‘‘conflicting benefits and harms’’ (level I) according to the grading proposed by United States Preventive Services Task Force [9]. These recommendations can be tailored according to the local expertise and context in the management of individual patients.

Section snippets

Consensus statements

A. “Symptomatic patients as follows are indication for treatment”: (level B).

  • Pancreatic involvement: e.g., obstructive jaundice, abdominal pain, back pain.

  • Other organ involvement (OOI): e.g., Jaundice due to bile duct stricture.

B. “Asymptomatic patients as follows are indication for treatment”: (level B).

  • Pancreatic: Persistent pancreatic mass on imaging.

  • OOI: Persistent liver test abnormalities in a patient with associated IgG4-related sclerosing cholangitis (IgG4-SC).

Description

As some AIP patients (about

Conclusion

The present consensus statements are based on the available evidence, and eastern and western experts' opinions to find standard treatment of AIP worldwide. These recommendations can be tailored according to the local expertise and context in the management of individual patients. Further studies, especially RCT studies for treatment, in addition to validations of consensus recommendations are needed.

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