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Autoimmune pancreatitis type 1

MedGen UID:
927912
•Concept ID:
C4302243
•
Disease or Syndrome
Synonyms: IgG4 related Pancreatitis; IgG4-related Pancreatitides; IgG4-related Pancreatitis; Pancreatitis, IgG4-related; Type 1 AIP; Type 1 Autoimmune Pancreatitis
SNOMED CT: Autoimmune pancreatitis type 1 (722872000); Type 1 IgG4 related autoimmune pancreatitis (722872000)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0017227
Orphanet: ORPHA280302

Definition

Type 1 autoimmune pancreatitis is a form of autoimmune pancreatitis seen in elderly males (>60 years) and presenting with abdominal pain, steatorrhea, obstructive jaundice and other organ (bile duct, kidneys and retroperitoneum) involvement. It is thought to be due to an immunoglobulin G4 (IgG4)-associated systemic disease. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAutoimmune pancreatitis type 1

Professional guidelines

PubMed

Type 1 Autoimmune Pancreatitis in Europe: Clinical Profile and Response to Treatment.
Overbeek KA, Poulsen JL, Lanzillotta M, Vinge-Holmquist O, Macinga P, Demirci AF, Sindhunata DP, Backhus J, Algül H, Buijs J, Levy P, Kiriukova M, Goni E, Hollenbach M, Miksch RC, Kunovsky L, Vujasinovic M, Nikolic S, Dickerson L, Hirth M, Neurath MF, Zumblick M, Vila J, Jalal M, Beyer G, Frost F, Carrara S, Kala Z, Jabandziev P, Sisman G, Akyuz F, Capurso G, Falconi M, Arlt A, Vleggaar FP, Barresi L, Greenhalf B, Czakó L, Hegyi P, Hopper A, Nayar MK, Gress TM, Vitali F, Schneider A, Halloran CM, Trna J, Okhlobystin AV, Dagna L, Cahen DL, Bordin D, Rebours V, Mayerle J, Kahraman A, Rasch S, Culver E, Kleger A, Martínez-Moneo E, Røkke O, Hucl T, Olesen SS, Bruno MJ, Della-Torre E, Beuers U, Löhr JM, Rosendahl J; PrescrAIP Study Group
Clin Gastroenterol Hepatol 2024 May;22(5):994-1004.e10. Epub 2024 Jan 5 doi: 10.1016/j.cgh.2023.12.010. PMID: 38184096
Autoimmune Pancreatitis: From Pathogenesis to Treatment.
Nista EC, De Lucia SS, Manilla V, Schepis T, Pellegrino A, Ojetti V, Pignataro G, Zileri Dal Verme L, Franceschi F, Gasbarrini A, Candelli M
Int J Mol Sci 2022 Oct 21;23(20) doi: 10.3390/ijms232012667. PMID: 36293522Free PMC Article
European Guideline on IgG4-related digestive disease - UEG and SGF evidence-based recommendations.
Löhr JM, Beuers U, Vujasinovic M, Alvaro D, Frøkjær JB, Buttgereit F, Capurso G, Culver EL, de-Madaria E, Della-Torre E, Detlefsen S, Dominguez-Muñoz E, Czubkowski P, Ewald N, Frulloni L, Gubergrits N, Duman DG, Hackert T, Iglesias-Garcia J, Kartalis N, Laghi A, Lammert F, Lindgren F, Okhlobystin A, Oracz G, Parniczky A, Mucelli RMP, Rebours V, Rosendahl J, Schleinitz N, Schneider A, van Bommel EF, Verbeke CS, Vullierme MP, Witt H; UEG guideline working group
United European Gastroenterol J 2020 Jul;8(6):637-666. Epub 2020 Jun 18 doi: 10.1177/2050640620934911. PMID: 32552502Free PMC Article

Recent clinical studies

Etiology

Efficacy and safety of rituximab in autoimmune pancreatitis type 1: our experiences and systematic review of the literature.
Nikolic S, Panic N, Hintikka ES, Dani L, Rutkowski W, Hedström A, Steiner C, Löhr JM, Vujasinovic M
Scand J Gastroenterol 2021 Nov;56(11):1355-1362. Epub 2021 Aug 19 doi: 10.1080/00365521.2021.1963837. PMID: 34410885
European Guideline on IgG4-related digestive disease - UEG and SGF evidence-based recommendations.
Löhr JM, Beuers U, Vujasinovic M, Alvaro D, Frøkjær JB, Buttgereit F, Capurso G, Culver EL, de-Madaria E, Della-Torre E, Detlefsen S, Dominguez-Muñoz E, Czubkowski P, Ewald N, Frulloni L, Gubergrits N, Duman DG, Hackert T, Iglesias-Garcia J, Kartalis N, Laghi A, Lammert F, Lindgren F, Okhlobystin A, Oracz G, Parniczky A, Mucelli RMP, Rebours V, Rosendahl J, Schleinitz N, Schneider A, van Bommel EF, Verbeke CS, Vullierme MP, Witt H; UEG guideline working group
United European Gastroenterol J 2020 Jul;8(6):637-666. Epub 2020 Jun 18 doi: 10.1177/2050640620934911. PMID: 32552502Free PMC Article
Immunoglobulin G subtypes-1 and 2 differentiate immunoglobulin G4-associated sclerosing cholangitis from primary sclerosing cholangitis.
Vujasinovic M, Maier P, Maetzel H, Valente R, Pozzi-Mucelli R, Moro CF, Haas SL, Said K, Verbeke CS, Maisonneuve P, Löhr JM
United European Gastroenterol J 2020 Jun;8(5):584-593. Epub 2020 Apr 29 doi: 10.1177/2050640620916027. PMID: 32349627Free PMC Article
Risk factors and treatment of relapses in autoimmune pancreatitis: Rituximab is safe and effective.
Soliman H, Vullierme MP, Maire F, Hentic O, Ruszniewski P, Lévy P, Rebours V
United European Gastroenterol J 2019 Oct;7(8):1073-1083. Epub 2019 Jul 3 doi: 10.1177/2050640619862459. PMID: 31662864Free PMC Article
Efficacy of Steroid Pulse Therapy for Autoimmune Pancreatitis Type 1: A Retrospective Study.
Sugimoto M, Takagi T, Suzuki R, Konno N, Watanabe K, Nakamura J, Kikuchi H, Waragai Y, Asama H, Takasumi M, Hikichi T, Watanabe H, Obara K, Ohira H
PLoS One 2015;10(9):e0138604. Epub 2015 Sep 18 doi: 10.1371/journal.pone.0138604. PMID: 26381760Free PMC Article

Diagnosis

Autoimmune pancreatitis – a surgical mistake?
Havlůj L, Whitley A, Hajer J, Hráček T, Gürlich R
Rozhl Chir 2022 Winter;101(11):540-544. doi: 10.33699/PIS.2022.101.11.540-544. PMID: 36717262
Autoimmune Pancreatitis: From Pathogenesis to Treatment.
Nista EC, De Lucia SS, Manilla V, Schepis T, Pellegrino A, Ojetti V, Pignataro G, Zileri Dal Verme L, Franceschi F, Gasbarrini A, Candelli M
Int J Mol Sci 2022 Oct 21;23(20) doi: 10.3390/ijms232012667. PMID: 36293522Free PMC Article
European Guideline on IgG4-related digestive disease - UEG and SGF evidence-based recommendations.
Löhr JM, Beuers U, Vujasinovic M, Alvaro D, Frøkjær JB, Buttgereit F, Capurso G, Culver EL, de-Madaria E, Della-Torre E, Detlefsen S, Dominguez-Muñoz E, Czubkowski P, Ewald N, Frulloni L, Gubergrits N, Duman DG, Hackert T, Iglesias-Garcia J, Kartalis N, Laghi A, Lammert F, Lindgren F, Okhlobystin A, Oracz G, Parniczky A, Mucelli RMP, Rebours V, Rosendahl J, Schleinitz N, Schneider A, van Bommel EF, Verbeke CS, Vullierme MP, Witt H; UEG guideline working group
United European Gastroenterol J 2020 Jul;8(6):637-666. Epub 2020 Jun 18 doi: 10.1177/2050640620934911. PMID: 32552502Free PMC Article
Risk factors and treatment of relapses in autoimmune pancreatitis: Rituximab is safe and effective.
Soliman H, Vullierme MP, Maire F, Hentic O, Ruszniewski P, Lévy P, Rebours V
United European Gastroenterol J 2019 Oct;7(8):1073-1083. Epub 2019 Jul 3 doi: 10.1177/2050640619862459. PMID: 31662864Free PMC Article
Recent advances in autoimmune pancreatitis: type 1 and type 2.
Kamisawa T, Chari ST, Lerch MM, Kim MH, Gress TM, Shimosegawa T
Gut 2013 Sep;62(9):1373-80. Epub 2013 Jun 8 doi: 10.1136/gutjnl-2012-304224. PMID: 23749606

Therapy

Autoimmune Pancreatitis: From Pathogenesis to Treatment.
Nista EC, De Lucia SS, Manilla V, Schepis T, Pellegrino A, Ojetti V, Pignataro G, Zileri Dal Verme L, Franceschi F, Gasbarrini A, Candelli M
Int J Mol Sci 2022 Oct 21;23(20) doi: 10.3390/ijms232012667. PMID: 36293522Free PMC Article
Efficacy and safety of rituximab in autoimmune pancreatitis type 1: our experiences and systematic review of the literature.
Nikolic S, Panic N, Hintikka ES, Dani L, Rutkowski W, Hedström A, Steiner C, Löhr JM, Vujasinovic M
Scand J Gastroenterol 2021 Nov;56(11):1355-1362. Epub 2021 Aug 19 doi: 10.1080/00365521.2021.1963837. PMID: 34410885
European Guideline on IgG4-related digestive disease - UEG and SGF evidence-based recommendations.
Löhr JM, Beuers U, Vujasinovic M, Alvaro D, Frøkjær JB, Buttgereit F, Capurso G, Culver EL, de-Madaria E, Della-Torre E, Detlefsen S, Dominguez-Muñoz E, Czubkowski P, Ewald N, Frulloni L, Gubergrits N, Duman DG, Hackert T, Iglesias-Garcia J, Kartalis N, Laghi A, Lammert F, Lindgren F, Okhlobystin A, Oracz G, Parniczky A, Mucelli RMP, Rebours V, Rosendahl J, Schleinitz N, Schneider A, van Bommel EF, Verbeke CS, Vullierme MP, Witt H; UEG guideline working group
United European Gastroenterol J 2020 Jul;8(6):637-666. Epub 2020 Jun 18 doi: 10.1177/2050640620934911. PMID: 32552502Free PMC Article
Efficacy of Steroid Pulse Therapy for Autoimmune Pancreatitis Type 1: A Retrospective Study.
Sugimoto M, Takagi T, Suzuki R, Konno N, Watanabe K, Nakamura J, Kikuchi H, Waragai Y, Asama H, Takasumi M, Hikichi T, Watanabe H, Obara K, Ohira H
PLoS One 2015;10(9):e0138604. Epub 2015 Sep 18 doi: 10.1371/journal.pone.0138604. PMID: 26381760Free PMC Article
Recent advances in autoimmune pancreatitis: type 1 and type 2.
Kamisawa T, Chari ST, Lerch MM, Kim MH, Gress TM, Shimosegawa T
Gut 2013 Sep;62(9):1373-80. Epub 2013 Jun 8 doi: 10.1136/gutjnl-2012-304224. PMID: 23749606

Prognosis

Küttner's Tumor and Autoimmune Pancreatitis as Metachronous Manifestations of IgG4-Related Disease.
Bathobakae L, Bashir R, Farhan H, Shahid A, Villegas K, Melki G, Amer K, Cavanagh Y
J Investig Med High Impact Case Rep 2024 Jan-Dec;12:23247096241286368. doi: 10.1177/23247096241286368. PMID: 39369317Free PMC Article
Immunoglobulin G subtypes-1 and 2 differentiate immunoglobulin G4-associated sclerosing cholangitis from primary sclerosing cholangitis.
Vujasinovic M, Maier P, Maetzel H, Valente R, Pozzi-Mucelli R, Moro CF, Haas SL, Said K, Verbeke CS, Maisonneuve P, Löhr JM
United European Gastroenterol J 2020 Jun;8(5):584-593. Epub 2020 Apr 29 doi: 10.1177/2050640620916027. PMID: 32349627Free PMC Article
Relapse patterns and predictors of IgG4-related diseases involved with autoimmune pancreatitis: A single-center retrospective study of 115 patients.
Miki M, Fujimori N, Oono T, Kawabe K, Ohno A, Matsumoto K, Teramatsu K, Tachibana Y, Ogawa Y
J Dig Dis 2019 Mar;20(3):152-158. doi: 10.1111/1751-2980.12708. PMID: 30701685
Distinct pathophysiological cytokine profiles for discrimination between autoimmune pancreatitis, chronic pancreatitis, and pancreatic ductal adenocarcinoma.
Ghassem-Zadeh S, Gaida MM, Szanyi S, Acha-Orbea H, Frossard JL, Hinz U, Hackert T, Strobel O, Felix K
J Transl Med 2017 Jun 2;15(1):126. doi: 10.1186/s12967-017-1227-3. PMID: 28578701Free PMC Article
Efficacy of Steroid Pulse Therapy for Autoimmune Pancreatitis Type 1: A Retrospective Study.
Sugimoto M, Takagi T, Suzuki R, Konno N, Watanabe K, Nakamura J, Kikuchi H, Waragai Y, Asama H, Takasumi M, Hikichi T, Watanabe H, Obara K, Ohira H
PLoS One 2015;10(9):e0138604. Epub 2015 Sep 18 doi: 10.1371/journal.pone.0138604. PMID: 26381760Free PMC Article

Clinical prediction guides

Immunoglobulin G subtypes-1 and 2 differentiate immunoglobulin G4-associated sclerosing cholangitis from primary sclerosing cholangitis.
Vujasinovic M, Maier P, Maetzel H, Valente R, Pozzi-Mucelli R, Moro CF, Haas SL, Said K, Verbeke CS, Maisonneuve P, Löhr JM
United European Gastroenterol J 2020 Jun;8(5):584-593. Epub 2020 Apr 29 doi: 10.1177/2050640620916027. PMID: 32349627Free PMC Article
Novel Autoantibody Signatures in Sera of Patients with Pancreatic Cancer, Chronic Pancreatitis and Autoimmune Pancreatitis: A Protein Microarray Profiling Approach.
Ghassem-Zadeh S, Hufnagel K, Bauer A, Frossard JL, Yoshida M, Kutsumi H, Acha-Orbea H, Neulinger-Muñoz M, Vey J, Eckert C, Strobel O, Hoheisel JD, Felix K
Int J Mol Sci 2020 Mar 31;21(7) doi: 10.3390/ijms21072403. PMID: 32244327Free PMC Article
Relapse patterns and predictors of IgG4-related diseases involved with autoimmune pancreatitis: A single-center retrospective study of 115 patients.
Miki M, Fujimori N, Oono T, Kawabe K, Ohno A, Matsumoto K, Teramatsu K, Tachibana Y, Ogawa Y
J Dig Dis 2019 Mar;20(3):152-158. doi: 10.1111/1751-2980.12708. PMID: 30701685
Distinct pathophysiological cytokine profiles for discrimination between autoimmune pancreatitis, chronic pancreatitis, and pancreatic ductal adenocarcinoma.
Ghassem-Zadeh S, Gaida MM, Szanyi S, Acha-Orbea H, Frossard JL, Hinz U, Hackert T, Strobel O, Felix K
J Transl Med 2017 Jun 2;15(1):126. doi: 10.1186/s12967-017-1227-3. PMID: 28578701Free PMC Article

Recent systematic reviews

Efficacy and safety of rituximab in autoimmune pancreatitis type 1: our experiences and systematic review of the literature.
Nikolic S, Panic N, Hintikka ES, Dani L, Rutkowski W, Hedström A, Steiner C, Löhr JM, Vujasinovic M
Scand J Gastroenterol 2021 Nov;56(11):1355-1362. Epub 2021 Aug 19 doi: 10.1080/00365521.2021.1963837. PMID: 34410885
IgG4-related disease: a systematic review of this unrecognized disease in pediatrics.
Karim F, Loeffen J, Bramer W, Westenberg L, Verdijk R, van Hagen M, van Laar J
Pediatr Rheumatol Online J 2016 Mar 25;14(1):18. doi: 10.1186/s12969-016-0079-3. PMID: 27012661Free PMC Article

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