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Autoimmune hepatitis(AIH)

MedGen UID:
1666753
Concept ID:
C4721555
Disease or Syndrome
Synonyms: AIH; Autoimmune chronic hepatitis
SNOMED CT: Autoimmune hepatitis (408335007)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0016264
Orphanet: ORPHA2137

Definition

A rare liver disease characterized by immune-mediated, acute or chronic liver inflammation, clinically presenting as cryptogenic hepatitis, with interface hepatitis on histological examination, elevated serum aminotransferase levels, and hypergammaglobulinemia / elevated immunoglobulin G, in the presence or absence of specific circulating autoantibodies. Patients may be asymptomatic, chronically ill, or present with acute liver failure. Concurrent autoimmune diseases are frequently observed. [from ORDO]

Professional guidelines

PubMed

Muratori L, Lohse AW, Lenzi M
BMJ 2023 Feb 6;380:e070201. doi: 10.1136/bmj-2022-070201. PMID: 36746473
Komori A
Clin Mol Hepatol 2021 Jan;27(1):58-69. Epub 2020 Dec 10 doi: 10.3350/cmh.2020.0189. PMID: 33291862Free PMC Article
Kwo PY, Cohen SM, Lim JK
Am J Gastroenterol 2017 Jan;112(1):18-35. Epub 2016 Dec 20 doi: 10.1038/ajg.2016.517. PMID: 27995906

Recent clinical studies

Etiology

Harrington C, Krishnan S, Mack CL, Cravedi P, Assis DN, Levitsky J
Hepatology 2022 Dec;76(6):1862-1879. Epub 2022 Jun 8 doi: 10.1002/hep.32591. PMID: 35611859Free PMC Article
Pape S, Snijders RJALM, Gevers TJG, Chazouilleres O, Dalekos GN, Hirschfield GM, Lenzi M, Trauner M, Manns MP, Vierling JM, Montano-Loza AJ, Lohse AW, Schramm C, Drenth JPH, Heneghan MA; International Autoimmune Hepatitis Group (IAIHG) collaborators(‡)
J Hepatol 2022 Apr;76(4):841-849. Epub 2022 Jan 20 doi: 10.1016/j.jhep.2021.12.041. PMID: 35066089
Terziroli Beretta-Piccoli B, Mieli-Vergani G, Vergani D
Clin Rev Allergy Immunol 2022 Oct;63(2):124-137. Epub 2021 Sep 7 doi: 10.1007/s12016-021-08888-9. PMID: 34491531Free PMC Article
Sucher E, Sucher R, Gradistanac T, Brandacher G, Schneeberger S, Berg T
J Immunol Res 2019;2019:9437043. Epub 2019 Nov 25 doi: 10.1155/2019/9437043. PMID: 31886312Free PMC Article
Nat Rev Dis Primers 2018 Apr 12;4:18018. doi: 10.1038/nrdp.2018.18. PMID: 29644995

Diagnosis

Muratori L, Lohse AW, Lenzi M
BMJ 2023 Feb 6;380:e070201. doi: 10.1136/bmj-2022-070201. PMID: 36746473
Tan CK, Ho D, Wang LM, Kumar R
World J Gastroenterol 2022 Jun 28;28(24):2654-2666. doi: 10.3748/wjg.v28.i24.2654. PMID: 35979160Free PMC Article
Olivas I, Rodríguez-Tajes S, Londoño MC
Med Clin (Barc) 2022 Sep 23;159(6):289-298. Epub 2022 Jun 7 doi: 10.1016/j.medcli.2022.04.004. PMID: 35688746
Komori A
Clin Mol Hepatol 2021 Jan;27(1):58-69. Epub 2020 Dec 10 doi: 10.3350/cmh.2020.0189. PMID: 33291862Free PMC Article
Sucher E, Sucher R, Gradistanac T, Brandacher G, Schneeberger S, Berg T
J Immunol Res 2019;2019:9437043. Epub 2019 Nov 25 doi: 10.1155/2019/9437043. PMID: 31886312Free PMC Article

Therapy

Olivas I, Rodríguez-Tajes S, Londoño MC
Med Clin (Barc) 2022 Sep 23;159(6):289-298. Epub 2022 Jun 7 doi: 10.1016/j.medcli.2022.04.004. PMID: 35688746
Komori A
Clin Mol Hepatol 2021 Jan;27(1):58-69. Epub 2020 Dec 10 doi: 10.3350/cmh.2020.0189. PMID: 33291862Free PMC Article
Tanaka A
Gut Liver 2020 Jul 15;14(4):430-438. doi: 10.5009/gnl19261. PMID: 32301319Free PMC Article
Pape S, Schramm C, Gevers TJ
United European Gastroenterol J 2019 Nov;7(9):1156-1163. Epub 2019 Aug 25 doi: 10.1177/2050640619872408. PMID: 31700628Free PMC Article
Terziroli Beretta-Piccoli B, Mieli-Vergani G, Vergani D
World J Gastroenterol 2017 Sep 7;23(33):6030-6048. doi: 10.3748/wjg.v23.i33.6030. PMID: 28970719Free PMC Article

Prognosis

Mroskowiak A, Suleja A, Stec M, Kuczmik W, Migacz M, Holecki M
Medicina (Kaunas) 2022 Jul 4;58(7) doi: 10.3390/medicina58070896. PMID: 35888614Free PMC Article
Harrington C, Krishnan S, Mack CL, Cravedi P, Assis DN, Levitsky J
Hepatology 2022 Dec;76(6):1862-1879. Epub 2022 Jun 8 doi: 10.1002/hep.32591. PMID: 35611859Free PMC Article
Katsumi T, Ueno Y
Liver Int 2022 Aug;42(9):2015-2022. Epub 2022 Jan 10 doi: 10.1111/liv.15155. PMID: 34990076
Trivedi PJ, Hirschfield GM
Gut 2021 Oct;70(10):1989-2003. Epub 2021 Jul 15 doi: 10.1136/gutjnl-2020-322362. PMID: 34266966
Schmid P, Rugo HS, Adams S, Schneeweiss A, Barrios CH, Iwata H, Diéras V, Henschel V, Molinero L, Chui SY, Maiya V, Husain A, Winer EP, Loi S, Emens LA; IMpassion130 Investigators
Lancet Oncol 2020 Jan;21(1):44-59. Epub 2019 Nov 27 doi: 10.1016/S1470-2045(19)30689-8. PMID: 31786121

Clinical prediction guides

Terziroli Beretta-Piccoli B, Mieli-Vergani G, Vergani D
Clin Rev Allergy Immunol 2022 Oct;63(2):124-137. Epub 2021 Sep 7 doi: 10.1007/s12016-021-08888-9. PMID: 34491531Free PMC Article
Komori A
Clin Mol Hepatol 2021 Jan;27(1):58-69. Epub 2020 Dec 10 doi: 10.3350/cmh.2020.0189. PMID: 33291862Free PMC Article
Tanaka A
Gut Liver 2020 Jul 15;14(4):430-438. doi: 10.5009/gnl19261. PMID: 32301319Free PMC Article
Sucher E, Sucher R, Gradistanac T, Brandacher G, Schneeberger S, Berg T
J Immunol Res 2019;2019:9437043. Epub 2019 Nov 25 doi: 10.1155/2019/9437043. PMID: 31886312Free PMC Article
Sharma S, Khalili K, Nguyen GC
World J Gastroenterol 2014 Dec 7;20(45):16820-30. doi: 10.3748/wjg.v20.i45.16820. PMID: 25492996Free PMC Article

Recent systematic reviews

Jensen AH, Winther-Sørensen M, Burisch J, Bergquist A, Ytting H, Gluud LL, Wewer Albrechtsen NJ
Liver Int 2023 Nov;43(11):2479-2491. Epub 2023 Sep 26 doi: 10.1111/liv.15720. PMID: 37752719
Pape S, Snijders RJALM, Gevers TJG, Chazouilleres O, Dalekos GN, Hirschfield GM, Lenzi M, Trauner M, Manns MP, Vierling JM, Montano-Loza AJ, Lohse AW, Schramm C, Drenth JPH, Heneghan MA; International Autoimmune Hepatitis Group (IAIHG) collaborators(‡)
J Hepatol 2022 Apr;76(4):841-849. Epub 2022 Jan 20 doi: 10.1016/j.jhep.2021.12.041. PMID: 35066089
Kaegi C, Wuest B, Schreiner J, Steiner UC, Vultaggio A, Matucci A, Crowley C, Boyman O
Front Immunol 2019;10:1990. Epub 2019 Sep 6 doi: 10.3389/fimmu.2019.01990. PMID: 31555262Free PMC Article
Mieli-Vergani G, Vergani D, Baumann U, Czubkowski P, Debray D, Dezsofi A, Fischler B, Gupte G, Hierro L, Indolfi G, Jahnel J, Smets F, Verkade HJ, Hadžić N
J Pediatr Gastroenterol Nutr 2018 Feb;66(2):345-360. doi: 10.1097/MPG.0000000000001801. PMID: 29356770
Terziroli Beretta-Piccoli B, Mieli-Vergani G, Vergani D
World J Gastroenterol 2017 Sep 7;23(33):6030-6048. doi: 10.3748/wjg.v23.i33.6030. PMID: 28970719Free PMC Article

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