title: Long-term outcomes of autoimmune pancreatitis: a multicentre, international analysis.
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.
  
  
  METHODS
  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.
authors.affiliation: Division of Gastroenterology and Hepatology, Mayo Clinic, , Rochester, Minnesota, USA.
authors.forename: Phil A
authors.initials: PA
authors.lastname: Hart
authors.forename: Terumi
authors.initials: T
authors.lastname: Kamisawa
authors.forename: William R
authors.initials: WR
authors.lastname: Brugge
authors.forename: Jae Bock
authors.initials: JB
authors.lastname: Chung
authors.forename: Emma L
authors.initials: EL
authors.lastname: Culver
authors.forename: László
authors.initials: L
authors.lastname: Czakó
authors.forename: Luca
authors.initials: L
authors.lastname: Frulloni
authors.forename: Vay Liang W
authors.initials: VL
authors.lastname: Go
authors.forename: Thomas M
authors.initials: TM
authors.lastname: Gress
authors.forename: Myung-Hwan
authors.initials: MH
authors.lastname: Kim
authors.forename: Shigeyuki
authors.initials: S
authors.lastname: Kawa
authors.forename: Kyu Taek
authors.initials: KT
authors.lastname: Lee
authors.forename: Markus M
authors.initials: MM
authors.lastname: Lerch
authors.forename: Wei-Chih
authors.initials: WC
authors.lastname: Liao
authors.forename: Matthias
authors.initials: M
authors.lastname: Löhr
authors.forename: Kazuichi
authors.initials: K
authors.lastname: Okazaki
authors.forename: Ji Kon
authors.initials: JK
authors.lastname: Ryu
authors.forename: Nicolas
authors.initials: N
authors.lastname: Schleinitz
authors.forename: Kyoko
authors.initials: K
authors.lastname: Shimizu
authors.forename: Tooru
authors.initials: T
authors.lastname: Shimosegawa
authors.forename: Roy
authors.initials: R
authors.lastname: Soetikno
authors.forename: George
authors.initials: G
authors.lastname: Webster
authors.forename: Dhiraj
authors.initials: D
authors.lastname: Yadav
authors.forename: Yoh
authors.initials: Y
authors.lastname: Zen
authors.forename: Suresh T
authors.initials: ST
authors.lastname: Chari
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001327:Autoimmune Diseases / Q000188:drug therapy / Q000473:pathology / Q000601:surgery / Q000628:therapy*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D050500:Pancreatitis, Chronic / Q000188:drug therapy / Q000473:pathology / Q000601:surgery / Q000628:therapy*; D011239:Prednisolone / Q000627:therapeutic use; D012074:Remission Induction; D055502:Secondary Prevention; D016896:Treatment Outcome
chemical_list: D000893:Anti-Inflammatory Agents; D011239:Prednisolone