title: Clinical profile of autoimmune pancreatitis and its histological subtypes: an international multicenter survey.
abstract: OBJECTIVE
  The objective of this study was to clarify the clinical and pathophysiological characteristics of autoimmune pancreatitis (AIP) and its subtypes (lymphoplasmacytic sclerosing pancreatitis [LPSP] and idiopathic duct-centric pancreatitis [IDCP]) seen around the world.
  
  
  METHODS
  An international multicenter survey of AIP was conducted in 15 institutes from 8 countries. We compared clinical and pathologic profiles of AIP (n = 731) and the clinical profiles of LPSP (n = 204) and IDCP (n = 64) patients.
  
  
  RESULTS
  Patients with LPSP were approximately 16 years older than IDCP patients. Obstructive jaundice was a more frequent presentation in LPSP versus IDCP (75% vs 47%, P < 0.001), whereas abdominal pain (41% vs 68%, P < 0.001) and acute pancreatitis (5% vs 34%, P < 0.001) were more frequent in IDCP patients. Patients with LPSP were more likely to have diffuse swelling of the pancreas (40% vs 25%, P = 0.037) and elevated serum IgG4 levels (63% vs 23%, P < 0.001) but less likely to be associated with ulcerative colitis (1% vs 16%, P < 0.001). Clinical profiles of non-histologically confirmed AIP from Asia, the United States, and United Kingdom corresponded with that of LPSP, whereas those from Italy and Germany suggested a mixture of LPSP and IDCP.
  
  
  CONCLUSIONS
  Autoimmune pancreatitis is seen all around the world, with regional differences in the pathologic and clinical features. Lymphoplasmacytic sclerosing pancreatitis and IDCP have distinct clinical profiles.
authors: Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan. kamisawa@cick.jp
authors: Terumi
authors: T
authors: Kamisawa
authors: Suresh T
authors: ST
authors: Chari
authors: Samuel A
authors: SA
authors: Giday
authors: Myung-Hwan
authors: MH
authors: Kim
authors: Jae Bock
authors: JB
authors: Chung
authors: Kyu Taek
authors: KT
authors: Lee
authors: Jens
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authors: Werner
authors: Frank
authors: F
authors: Bergmann
authors: Markus M
authors: MM
authors: Lerch
authors: Julia
authors: J
authors: Mayerle
authors: Tilman
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authors: Pickartz
authors: Matthias
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authors: Lohr
authors: Alexander
authors: A
authors: Schneider
authors: Luca
authors: L
authors: Frulloni
authors: George J M
authors: GJ
authors: Webster
authors: D Nageshwar
authors: DN
authors: Reddy
authors: Wei-Chih
authors: WC
authors: Liao
authors: Hsiu-Po
authors: HP
authors: Wang
authors: Kazuichi
authors: K
authors: Okazaki
authors: Tooru
authors: T
authors: Shimosegawa
authors: Guenter
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authors: Kloeppel
authors: Vay Liang W
authors: VL
authors: Go
mesh_terms: D015746:Abdominal Pain / Q000209:etiology; D000328:Adult; D000368:Aged; D001327:Autoimmune Diseases / Q000145:classification / Q000150:complications / Q000473:pathology*; D003625:Data Collection; D005260:Female; D006801:Humans; D015212:Inflammatory Bowel Diseases / Q000150:complications; D041781:Jaundice, Obstructive / Q000209:etiology; D008297:Male; D008875:Middle Aged; D010195:Pancreatitis / Q000145:classification / Q000150:complications / Q000473:pathology*; D012189:Retrospective Studies; D012598:Sclerosis; D013256:Steroids / Q000627:therapeutic use
chemical_list: D013256:Steroids