title: IgG4 related disease - a retrospective descriptive study highlighting Canadian experiences in diagnosis and management.
abstract: BACKGROUND
  Appreciating the utility of published diagnostic criteria for autoimmune pancreatitis, when compared to the characteristics of patients clinically managed as having disease, informs and refines ongoing clinical practice.
  
  
  METHODS
  Comparative retrospective descriptive evaluation of patients with autoimmune pancreatitis including dedicated radiology review.
  
  
  RESULTS
  66 subjects with radiographic OR clinical features of autoimmune pancreatitis were initially identifiable (Male: n = 50), with 55 confirmed for evaluation. The most common presentation included pain (67%), weight loss (65%), and jaundice (62%). Diffuse enlargement of the pancreas was evident in 38%, whilst multifocal, focal, or atrophic changes were seen in 7%, 33% and 9% respectively. 13% had no pancreatic parenchymal involvement. Peripheral rim enhancement was seen in 23 patients (42%). Where discernible, disease was a) Sclerosing pancreatitis and cholangitis, n = 21; b) Sclerosing cholangitis, n = 9; c) Sclerosing pancreatitis, n = 4; d) Sclerosing pancreatitis and cholangitis with pancreatic pseudotumour, n = 7; e) Sclerosing cholangitis with hepatic pseudotumour, n = 3; f) Sclerosing pancreatitis with pancreatic pseudotumour, n = 1. 56% of the patients had systemic manifestations and the median serum IgG4 at diagnosis was 5.12 g/L. The Korean criteria identified most patients (82%) compared to HISORt (55%) or the Japan Pancreas Society (56%). The majority (HISORt 60%; Japan Pancreas Society 55%; Korean 58%) met diagnostic criterion by radiological findings and elevated serum IgG4. Treatment and response did not differ when stratified by diagnostic criteria.
  
  
  CONCLUSIONS
  Our descriptive and retrospective dataset confirms that in non-expert practice settings, autoimmune pancreatitis scoring systems with a focus on radiology and serology capture most patients who are clinically felt to have disease.
authors: Harshna
authors: H
authors: Patel
authors: Korosh
authors: K
authors: Khalili
authors: Kim Tae
authors: KT
authors: Kyoung
authors: Leyla
authors: L
authors: Yazdi
authors: Eric
authors: E
authors: Lee
authors: Gary
authors: G
authors: May
authors: Paul
authors: P
authors: Kortan
authors: Catalina
authors: C
authors: Coltescu
authors: Centre for Liver Research and NIHR Biomedical Research Unit, Institute of Biomedical Research, The Medical School, University of Birmingham, 5th Floor, Birmingham B15 2TT, UK. g.hirschfield@bham.ac.uk.
authors: Gideon M
authors: GM
authors: Hirschfield
mesh_terms: D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000276:immunology / Q000628:therapy; D002170:Canada; D015209:Cholangitis, Sclerosing / Q000175:diagnosis* / Q000276:immunology / Q000628:therapy; D015331:Cohort Studies; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D010195:Pancreatitis / Q000175:diagnosis* / Q000276:immunology / Q000628:therapy; D012189:Retrospective Studies
chemical_list: D007074:Immunoglobulin G