Ontology highlight
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.
SUBMITTER: Hart PA
PROVIDER: S-EPMC3862979 | biostudies-literature | 2013 Dec
REPOSITORIES: biostudies-literature
Hart Phil A PA Kamisawa Terumi T Brugge William R WR Chung Jae Bock JB Culver Emma L EL Czakó László L Frulloni Luca L Go Vay Liang W VL Gress Thomas M TM Kim Myung-Hwan MH Kawa Shigeyuki S Lee Kyu Taek KT Lerch Markus M MM Liao Wei-Chih WC Löhr Matthias M Okazaki Kazuichi K Ryu Ji Kon JK Schleinitz Nicolas N Shimizu Kyoko K Shimosegawa Tooru T Soetikno Roy R Webster George G Yadav Dhiraj D Zen Yoh Y Chari Suresh T ST
Gut 20121211 12
<h4>Objective</h4>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.<h4>Design</h4>23 institutions from 10 different countries participated in t ...[more]