title: Type 1 autoimmune pancreatitis and IgG4-related sclerosing cholangitis is associated with extrapancreatic organ failure, malignancy, and mortality in a prospective UK cohort.
abstract: OBJECTIVE
  Type I autoimmune pancreatitis (AIP) and IgG4-related sclerosing cholangitis (IgG4-related SC) are now recognized as components of a multisystem IgG4-related disease (IgG4-RD). We aimed to define the clinical course and long-term outcomes in patients with AIP/IgG4-SC recruited from two large UK tertiary referral centers.
  
  
  METHODS
  Data were collected from 115 patients identified between 2004 and 2013, and all were followed up prospectively from diagnosis for a median of 33 months (range 1-107), and evaluated for response to therapy, the development of multiorgan involvement, and malignancy. Comparisons were made with national UK statistics.
  
  
  RESULTS
  Although there was an initial response to steroids in 97%, relapse occurred in 50% of patients. IgG4-SC was an important predictor of relapse (P<0.01). Malignancy occurred in 11% shortly before or after the diagnosis of IgG4-RD, including three hepatopancreaticobiliary cancers. The risk of any cancer at diagnosis or during follow-up when compared with matched national statistics was increased (odds ratio=2.25, CI=1.12-3.94, P=0.02). Organ dysfunction occurred within the pancreas, liver, kidney, lung, and brain. Mortality occurred in 10% of patients during follow-up. The risk of death was increased compared with matched national statistics (odds ratio=2.07, CI=1.07-3.55, P=0.02).
  
  
  CONCLUSIONS
  Our findings suggest that AIP and IgG4-SC are associated with significant morbidity and mortality owing to extrapancreatic organ failure and malignancy. Detailed clinical evaluation for evidence of organ dysfunction and associated malignancy is required both at first presentation and during long-term follow-up.
authors.affiliation: UCL Institute for Liver and Digestive Health, University College London, London, UK.|Department of Gastroenterology and Hepatology, University College Hospital, London, UK.
authors.forename: Matthew T
authors.initials: MT
authors.lastname: Huggett
authors.affiliation: Translational Gastroenterology Unit, John Radcliffe Hospital, Oxford, UK.|NDM Oxford University, Oxford Martin School, Oxford University, Oxford, UK.
authors.forename: E L
authors.initials: EL
authors.lastname: Culver
authors.affiliation: Department of Gastroenterology and Hepatology, University College Hospital, London, UK.
authors.forename: M
authors.initials: M
authors.lastname: Kumar
authors.affiliation: Institute of Emerging Diseases, Oxford Martin School, Oxford University, Oxford, UK.
authors.forename: J M
authors.initials: JM
authors.lastname: Hurst
authors.affiliation: Department of Pathology, University College Hospital, London, UK.
authors.forename: M
authors.initials: M
authors.lastname: Rodriguez-Justo
authors.affiliation: Department of Gastroenterology and Hepatology, University College Hospital, London, UK.
authors.forename: M H
authors.initials: MH
authors.lastname: Chapman
authors.affiliation: Department of Gastroenterology and Hepatology, University College Hospital, London, UK.
authors.forename: G J
authors.initials: GJ
authors.lastname: Johnson
authors.affiliation: UCL Institute for Liver and Digestive Health, University College London, London, UK.|Department of Gastroenterology and Hepatology, University College Hospital, London, UK.
authors.forename: S P
authors.initials: SP
authors.lastname: Pereira
authors.affiliation: Translational Gastroenterology Unit, John Radcliffe Hospital, Oxford, UK.|NDM Oxford University, Oxford Martin School, Oxford University, Oxford, UK.
authors.forename: R W
authors.initials: RW
authors.lastname: Chapman
authors.affiliation: Department of Gastroenterology and Hepatology, University College Hospital, London, UK.
authors.forename: George J M
authors.initials: GJM
authors.lastname: Webster
authors.affiliation: Translational Gastroenterology Unit, John Radcliffe Hospital, Oxford, UK.|NDM Oxford University, Oxford Martin School, Oxford University, Oxford, UK.|Oxford NIHR BRC, Oxford University, Oxford, UK.
authors.forename: E
authors.initials: E
authors.lastname: Barnes
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001327:Autoimmune Diseases / Q000150:complications* / Q000401:mortality / Q000628:therapy; D001927:Brain Diseases / Q000453:epidemiology; D015209:Cholangitis, Sclerosing / Q000150:complications* / Q000401:mortality / Q000628:therapy; D005260:Female; D006801:Humans; D007074:Immunoglobulin G; D007674:Kidney Diseases / Q000453:epidemiology; D008107:Liver Diseases / Q000453:epidemiology; D008171:Lung Diseases / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D010195:Pancreatitis / Q000150:complications* / Q000401:mortality / Q000628:therapy; D011446:Prospective Studies; D012307:Risk Factors; D006113:United Kingdom; D055815:Young Adult
chemical_list: D007074:Immunoglobulin G