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Autoimmune hepatitis type 2

MedGen UID:
928832
Concept ID:
C4303163
Disease or Syndrome
Synonyms: AIH type 2; autoimmune hepatitis type 2; Autoimmune Hepatitis, Type 2; Type 2 AIH; type 2 AIH; Type 2 Autoimmune Hepatitis
SNOMED CT: Autoimmune hepatitis type 2 (721712002)
 
Monarch Initiative: MONDO:0021176
Orphanet: ORPHA563581

Definition

A form of autoimmune hepatitis characterized by clinical presentation as cryptogenic hepatitis, interface hepatitis on histological examination, elevated serum aminotransferase levels, hypergammaglobulinemia/elevated immunoglobulin G, and presence of circulating autoantibodies, specifically antibodies to liver kidney microsome type 1 (anti-LKM1) and anti-liver cytosol type 1 (anti-LC1) antibodies. The disease typically manifests in childhood or adolescence with an acute onset, often with acute liver failure. Long-term immunosuppression is usually required. Reported concurrent autoimmune diseases are autoimmune thyroiditis, diabetes mellitus, and vitiligo. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAutoimmune hepatitis type 2

Professional guidelines

PubMed

Ludz C, Stirnimann G, Semela D, Mertens J, Kremer AE, Filipowicz Sinnreich M, Sokollik C, Bernsmeier C, Bresson-Hadni S, McLin V, Rock N, Braegger C, Posovszky C, Müller P, Cremer M, De Gottardi A, Galante A, Furlano R, Righini-Grunder F, Becker B, Böhm S, Heyland K, Nydegger A, Limoni C, Vergani D, Mieli-Vergani G, Di Bartolomeo C, Cerny A, Terziroli Beretta-Piccoli B
Swiss Med Wkly 2023 Aug 31;153:40102. doi: 10.57187/smw.2023.40102. PMID: 37769636
Dalekos GN, Wedemeyer H, Obermayer-Straub P, Kayser A, Barut A, Frank H, Manns MP
J Hepatol 1999 Mar;30(3):366-75. doi: 10.1016/s0168-8278(99)80092-0. PMID: 10190716

Recent clinical studies

Etiology

Dragani TA
J Hepatol 2010 Feb;52(2):252-7. Epub 2009 Nov 24 doi: 10.1016/j.jhep.2009.11.015. PMID: 20022654
Mieli-Vergani G, Vergani D
Semin Liver Dis 2009 Aug;29(3):297-306. Epub 2009 Aug 12 doi: 10.1055/s-0029-1233529. PMID: 19676002
Bridoux-Henno L, Maggiore G, Johanet C, Fabre M, Vajro P, Dommergues JP, Reinert P, Bernard O
Clin Gastroenterol Hepatol 2004 Sep;2(9):825-30. doi: 10.1016/s1542-3565(04)00354-4. PMID: 15354284
Dalekos GN, Wedemeyer H, Obermayer-Straub P, Kayser A, Barut A, Frank H, Manns MP
J Hepatol 1999 Mar;30(3):366-75. doi: 10.1016/s0168-8278(99)80092-0. PMID: 10190716
Lenzi M, Ballardini G, Fusconi M, Cassani F, Selleri L, Volta U, Zauli D, Bianchi FB
Lancet 1990 Feb 3;335(8684):258-9. doi: 10.1016/0140-6736(90)90070-l. PMID: 1967721

Diagnosis

Christen U
Immunotherapy 2013 Mar;5(3):247-56. doi: 10.2217/imt.13.5. PMID: 23444954
Mieli-Vergani G, Vergani D
Semin Liver Dis 2009 Aug;29(3):297-306. Epub 2009 Aug 12 doi: 10.1055/s-0029-1233529. PMID: 19676002
Bridoux-Henno L, Maggiore G, Johanet C, Fabre M, Vajro P, Dommergues JP, Reinert P, Bernard O
Clin Gastroenterol Hepatol 2004 Sep;2(9):825-30. doi: 10.1016/s1542-3565(04)00354-4. PMID: 15354284
Kerkar N, Ma Y, Davies ET, Cheeseman P, Mieli-Vergani G, Vergani D
J Clin Pathol 2002 Dec;55(12):906-9. doi: 10.1136/jcp.55.12.906. PMID: 12461054Free PMC Article
Manns MP
J Viral Hepat 1997;4 Suppl 1:7-10. doi: 10.1111/j.1365-2893.1997.tb00154.x. PMID: 9097272

Therapy

Mele D, Bossi G, Maggiore G, Oliviero B, Mantovani S, Bonelli B, Mondelli MU, Varchetta S
Clin Immunol 2018 Mar;188:31-37. Epub 2017 Dec 9 doi: 10.1016/j.clim.2017.12.004. PMID: 29233784
Holder BS, Grant CR, Liberal R, Ma Y, Heneghan MA, Mieli-Vergani G, Vergani D, Longhi MS
J Autoimmun 2014 Sep;53:26-32. Epub 2014 Feb 22 doi: 10.1016/j.jaut.2014.02.001. PMID: 24566085
Christen U
Immunotherapy 2013 Mar;5(3):247-56. doi: 10.2217/imt.13.5. PMID: 23444954
Mieli-Vergani G, Vergani D
Semin Liver Dis 2009 Aug;29(3):297-306. Epub 2009 Aug 12 doi: 10.1055/s-0029-1233529. PMID: 19676002
Bridoux-Henno L, Maggiore G, Johanet C, Fabre M, Vajro P, Dommergues JP, Reinert P, Bernard O
Clin Gastroenterol Hepatol 2004 Sep;2(9):825-30. doi: 10.1016/s1542-3565(04)00354-4. PMID: 15354284

Prognosis

Mieli-Vergani G, Vergani D
Semin Liver Dis 2009 Aug;29(3):297-306. Epub 2009 Aug 12 doi: 10.1055/s-0029-1233529. PMID: 19676002
Granito A, Muratori L, Muratori P, Pappas G, Guidi M, Cassani F, Volta U, Ferri A, Lenzi M, Bianchi FB
J Clin Pathol 2006 Mar;59(3):280-4. doi: 10.1136/jcp.2005.027367. PMID: 16505279Free PMC Article
Dalekos GN, Wedemeyer H, Obermayer-Straub P, Kayser A, Barut A, Frank H, Manns MP
J Hepatol 1999 Mar;30(3):366-75. doi: 10.1016/s0168-8278(99)80092-0. PMID: 10190716
Manns MP, Obermayer-Straub P
J Viral Hepat 1997;4 Suppl 2:42-7. doi: 10.1111/j.1365-2893.1997.tb00179.x. PMID: 9429209
Manns MP, Obermayer-Straub P
Hepatology 1997 Oct;26(4):1054-66. doi: 10.1002/hep.510260438. PMID: 9328334

Clinical prediction guides

Mieli-Vergani G, Vergani D
Semin Liver Dis 2009 Aug;29(3):297-306. Epub 2009 Aug 12 doi: 10.1055/s-0029-1233529. PMID: 19676002
Vergani D, Mieli-Vergani G
Semin Liver Dis 2007 May;27(2):140-51. doi: 10.1055/s-2007-979467. PMID: 17520514
Mizutani T, Shinoda M, Tanaka Y, Kuno T, Hattori A, Usui T, Kuno N, Osaka T
Drug Metab Rev 2005;37(1):235-52. doi: 10.1081/dmr-200028798. PMID: 15747502
Dalekos GN, Wedemeyer H, Obermayer-Straub P, Kayser A, Barut A, Frank H, Manns MP
J Hepatol 1999 Mar;30(3):366-75. doi: 10.1016/s0168-8278(99)80092-0. PMID: 10190716
Manns MP, Obermayer-Straub P
J Viral Hepat 1997;4 Suppl 2:42-7. doi: 10.1111/j.1365-2893.1997.tb00179.x. PMID: 9429209

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