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Original Article
Long-term outcomes of autoimmune pancreatitis: a multicentre, international analysis
  1. Phil A Hart1,
  2. Terumi Kamisawa2,
  3. William R Brugge3,
  4. Jae Bock Chung4,
  5. Emma L Culver5,
  6. László Czakó6,
  7. Luca Frulloni7,
  8. Vay Liang W Go8,
  9. Thomas M Gress9,
  10. Myung-Hwan Kim10,
  11. Shigeyuki Kawa11,
  12. Kyu Taek Lee12,
  13. Markus M Lerch13,
  14. Wei-Chih Liao14,
  15. Matthias Löhr15,
  16. Kazuichi Okazaki16,
  17. Ji Kon Ryu17,
  18. Nicolas Schleinitz18,
  19. Kyoko Shimizu19,
  20. Tooru Shimosegawa20,
  21. Roy Soetikno21,
  22. George Webster22,
  23. Dhiraj Yadav23,
  24. Yoh Zen24,
  25. Suresh T Chari1
  1. 1Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota, USA
  2. 2Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan
  3. 3Department of Internal Medicine, GI Unit, Massachusetts General Hospital, Boston, Massachusetts, USA
  4. 4Department of Internal Medicine, Institute of Gastroenterology, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea
  5. 5Translational Gastroenterology Unit, John Radcliffe Hospital, Oxford, UK
  6. 6First Department of Internal Medicine, University of Szeged, Szeged, Hungary
  7. 7Department of Medicine, Biomedical and Surgical Sciences, University of Verona, Verona, Italy
  8. 8Center for Excellence in Pancreatic Disease, David Geffen School of Medicine at University of California, Los Angeles, California, USA
  9. 9Department of Gastroenterology, Endocrinology, Metabolism and Infectiology, Philipps University of Marburg, Marburg, Germany
  10. 10Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
  11. 11Center for Health, Safety and Environmental Management, Shinshu University, Matsumoto, Japan
  12. 12Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
  13. 13Department of Medicine A, University Medicine Greifswald, Greifswald, Germany
  14. 14Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan
  15. 15Department of Surgical Gastroenterology, Karolinska Institutet & Karolinska University Hospital, Stockholm, Sweden
  16. 16Department of Gastroenterology and Hepatology, Kansai Medical University, Osaka, Japan
  17. 17Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea
  18. 18Department of Internal Medicine, Aix-Marseille University, Marseille, France
  19. 19Department of Gastroenterology, Tokyo Women's Medical University, School of Medicine, Tokyo, Japan
  20. 20Division of Gastroenterology, Tohoku University Graduate School of Medicine, Sendai, Japan
  21. 21Department of Internal Medicine, Affiliated Stanford University, Palo Alto, California, USA
  22. 22Department of Gastroenterology, University College Hospital, London, UK
  23. 23Division of Gastroenterology, Hepatology and Nutrition, University of Pittsburgh,  Pennsylvania, USA
  24. 24Institute of Liver Studies, King's College Hospital, London, UK
  1. Correspondence to Dr Terumi Kamisawa, Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, Tokyo 113-8677, Japan; kamisawa{at}cick.jp

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.

  • Autoimmune Disease
  • Pancreatic Cancer
  • Pancreato-Biliary Disorders

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