Long-term outcomes of autoimmune pancreatitis: a multicentre, international analysis

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Abstract

Objective

Autoimmune pancreatitis (AIP) is a treatable form of chronic pancreatitis that has been increasingly recognised over the last decade. We set out to better understand the current burden of AIP at several academic institutions diagnosed using the International Consensus Diagnostic Criteria, and to describe long-term outcomes, including organs involved, treatments, relapse frequency and long-term sequelae.

Design

23 institutions from 10 different countries participated in this multinational analysis. A total of 1064 patients meeting the International Consensus Diagnostic Criteria for type 1 (n=978) or type 2 (n=86) AIP were included. Data regarding treatments, relapses and sequelae were obtained.

Results

The majority of patients with type 1 (99%) and type 2 (92%) AIP who were treated with steroids went into clinical remission. Most patients with jaundice required biliary stent placement (71% of type 1 and 77% of type 2 AIP). Relapses were more common in patients with type 1 (31%) versus type 2 AIP (9%, p<0.001), especially those with IgG4-related sclerosing cholangitis (56% vs 26%, p<0.001). Relapses typically occurred in the pancreas or biliary tree. Retreatment with steroids remained effective at inducing remission with or without alternative treatment, such as azathioprine. Pancreatic duct stones and cancer were uncommon sequelae in type 1 AIP and did not occur in type 2 AIP during the study period.

Conclusions

AIP is a global disease which uniformly displays a high response to steroid treatment and tendency to relapse in the pancreas and biliary tree. Potential long-term sequelae include pancreatic duct stones and malignancy, however they were uncommon during the study period and require additional follow-up. Additional studies investigating prevention and treatment of disease relapses are needed.

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Most cited references 16

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International consensus diagnostic criteria for autoimmune pancreatitis: guidelines of the International Association of Pancreatology.

Tooru Shimosegawa, Suresh T. Chari, Luca Frulloni … (2011)

To achieve the goal of developing international consensus diagnostic criteria (ICDC) for autoimmune pancreatitis (AIP). An international panel of experts met during the 14th Congress of the International Association of Pancreatology held in Fukuoka, Japan, from July 11 through 13, 2010. The proposed criteria represent a consensus opinion of the working group. Autoimmune pancreatitis was classified into types 1 and 2. The ICDC used 5 cardinal features of AIP, namely, imaging of pancreatic parenchyma and duct, serology, other organ involvement, pancreatic histology, and an optional criterion of response to steroid therapy. Each feature was categorized as level 1 and 2 findings depending on the diagnostic reliability. The diagnosis of type 1 and type 2 AIP can be definitive or probable, and in some cases, the distinction between the subtypes may not be possible (AIP-not otherwise specified). The ICDC for AIP were developed based on the agreement of an international panel of experts in the hope that they will promote worldwide recognition of AIP. The categorization of AIP into types 1 and 2 should be helpful for further clarification of the clinical features, pathogenesis, and natural history of these diseases.

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Standard steroid treatment for autoimmune pancreatitis.

T Kamisawa, T. Shimosegawa, K Okazaki … (2009)

To establish an appropriate steroid treatment regimen for autoimmune pancreatitis (AIP). A retrospective survey of AIP treatment was conducted in 17 centres in Japan. The main outcome measures were rate of remission and relapse. Of 563 patients with AIP, 459 (82%) received steroid treatment. The remission rate of steroid-treated AIP was 98%, which was significantly higher than that of patients without steroid treatment (74%, 77/104; p<0.001). Steroid treatment was given for obstructive jaundice (60%), abdominal pain (11%), associated extrapancreatic lesions except the biliary duct (11%), and diffuse enlargement of the pancreas (10%). There was no relationship between the period necessary to achieve remission and the initial dose (30 mg/day vs 40 mg/day) of prednisolone. Maintenance steroid treatment was given in 377 (82%) of 459 steroid-treated patients, and steroid treatment was stopped in 104 patients. The relapse rate of patients with AIP on maintenance treatment was 23% (63/273), which was significantly lower than that of patients who stopped maintenance treatment (34%, 35/104; p = 0.048). From the start of steroid treatment, 56% (55/99) relapsed within 1 year and 92% (91/99) relapsed within 3 years. Of the 89 relapsed patients, 83 (93%) received steroid re-treatment, and steroid re-treatment was effective in 97% of them. The major indication for steroid treatment in AIP is the presence of symptoms. An initial prednisolone dose of 0.6 mg/kg/day, is recommend, which is then reduced to a maintenance dose over a period of 3-6 months. Maintenance treatment with low-dose steroid reduces but dose not eliminate relapses.

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Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy.

Amaar Ghazale, Suresh T. Chari, Lizhi Zhang … (2008)

Immunoglobulin (Ig)G4-associated cholangitis (IAC) is the biliary manifestation of a steroid-responsive multisystem fibroinflammatory disorder in which affected organs have a characteristic lymphoplasmacytic infiltrate rich in IgG4-positive cells. We describe clinical features, treatment response, and predictors of relapse in IAC and compare relapse rates in IAC with intrapancreatic vs proximal bile duct strictures. We reviewed clinical, serologic, and imaging characteristics and treatment response in 53 IAC patients. IAC patients generally were older (mean age, 62 y) men (85%), presenting with obstructive jaundice (77%) associated with autoimmune pancreatitis (92%), increased serum IgG4 levels (74%), and abundant IgG4-positive cells in bile duct biopsy specimens (88%). At presentation, biliary strictures were confined to the intrapancreatic bile duct in 51%; the proximal extrahepatic/intrahepatic ducts were involved in 49%. Initial presentation was treated with steroids (n = 30; median follow-up period, 29.5 months), surgical resection (n = 18; median follow-up period, 58 months), or was conservative (n = 5; median follow-up period, 35 months). Relapses occurred in 53% after steroid withdrawal; 44% relapsed after surgery and were treated with steroids. The presence of proximal extrahepatic/intrahepatic strictures was predictive of relapse. Steroid therapy normalized liver enzyme levels in 61%; biliary stents could be removed in 17 of 18 patients. Fifteen patients treated with steroids for relapse after steroid withdrawal responded; 7 patients on additional immunomodulatory drugs remain in steroid-free remission (median follow-up period, 6 months). IAC should be suspected in unexplained biliary strictures associated with increased serum IgG4 and unexplained pancreatic disease. Relapses are common after steroid withdrawal, especially with proximal strictures. The role of immunomodulatory drugs for relapses needs further study.

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Author and article information

Journal

Journal ID (nlm-ta): Gut

Journal ID (iso-abbrev): Gut

Journal ID (hwp): gutjnl

Journal ID (publisher-id): gut

Title: Gut

Publisher: BMJ Publishing Group (BMA House, Tavistock Square, London, WC1H 9JR )

ISSN (Print): 0017-5749

ISSN (Electronic): 1468-3288

Publication date (Print): December 2013

Publication date (Electronic): 11 December 2012

Volume: 62

Issue: 12

Pages: 1771-1776

Affiliations

[1 ]Division of Gastroenterology and Hepatology, Mayo Clinic , Rochester, Minnesota, USA

[2 ]Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital , Tokyo, Japan

[3 ]Department of Internal Medicine, GI Unit, Massachusetts General Hospital , Boston, Massachusetts, USA

[4 ]Department of Internal Medicine, Institute of Gastroenterology, Severance Hospital, Yonsei University College of Medicine , Seoul, Korea

[5 ]Translational Gastroenterology Unit, John Radcliffe Hospital , Oxford, UK

[6 ]First Department of Internal Medicine, University of Szeged , Szeged, Hungary

[7 ]Department of Medicine, Biomedical and Surgical Sciences, University of Verona , Verona, Italy

[8 ]Center for Excellence in Pancreatic Disease, David Geffen School of Medicine at University of California , Los Angeles, California, USA

[9 ]Department of Gastroenterology, Endocrinology, Metabolism and Infectiology, Philipps University of Marburg , Marburg, Germany

[10 ]Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine , Seoul, Korea

[11 ]Center for Health, Safety and Environmental Management, Shinshu University , Matsumoto, Japan

[12 ]Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine , Seoul, Korea

[13 ]Department of Medicine A, University Medicine Greifswald , Greifswald, Germany

[14 ]Department of Internal Medicine, National Taiwan University Hospital , Taipei, Taiwan

[15 ]Department of Surgical Gastroenterology, Karolinska Institutet & Karolinska University Hospital , Stockholm, Sweden

[16 ]Department of Gastroenterology and Hepatology, Kansai Medical University , Osaka, Japan

[17 ]Department of Internal Medicine, Seoul National University College of Medicine , Seoul, Korea

[18 ]Department of Internal Medicine, Aix-Marseille University , Marseille, France

[19 ]Department of Gastroenterology, Tokyo Women's Medical University, School of Medicine , Tokyo, Japan

[20 ]Division of Gastroenterology, Tohoku University Graduate School of Medicine , Sendai, Japan

[21 ]Department of Internal Medicine, Affiliated Stanford University , Palo Alto, California, USA

[22 ]Department of Gastroenterology, University College Hospital , London, UK

[23 ]Division of Gastroenterology, Hepatology and Nutrition, University of Pittsburgh , Pennsylvania, USA

[24 ]Institute of Liver Studies, King's College Hospital , London, UK

Author notes

[Correspondence to ] Dr Terumi Kamisawa, Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, Tokyo 113-8677, Japan; kamisawa@ 123456cick.jp

Article

Publisher ID: gutjnl-2012-303617

DOI: 10.1136/gutjnl-2012-303617

PMC ID: 3862979

PubMed ID: 23232048

SO-VID: 29c9a374-d679-4137-ad73-f81668506794

License:

This is an Open Access article distributed in accordance with the terms of the Creative Commons Attribution (CC BY 3.0) license, which permits others to distribute, remix, adapt and build upon this work, for commercial use, provided the original work is properly cited. See: http://creativecommons.org/licenses/by/3.0/