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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
Jurado A, Cárdaba B, Jara P, Cuadrado P, Hierro L, de Andrés B, del Pozo V, Cortegano MI, Gallardo S, Camarena C, Bárcena R, Castañer JL, Alvarez R, Lahoz C, Palomino P
J Hepatol 1997 May;26(5):983-91. doi: 10.1016/s0168-8278(97)80106-7. PMID: 9186828
Obermayer-Straub P, Manns MP
Mol Biol Rep 1996;23(3-4):235-42. doi: 10.1007/BF00351174. PMID: 9112234
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
Invernizzi P, Alessio MG, Smyk DS, Lleo A, Sonzogni A, Fabris L, Candusso M, Bogdanos DP, Iorio R, Torre G
BMC Gastroenterol 2012 Jul 20;12:92. doi: 10.1186/1471-230X-12-92. PMID: 22816667Free PMC Article
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
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
Invernizzi P, Alessio MG, Smyk DS, Lleo A, Sonzogni A, Fabris L, Candusso M, Bogdanos DP, Iorio R, Torre G
BMC Gastroenterol 2012 Jul 20;12:92. doi: 10.1186/1471-230X-12-92. PMID: 22816667Free PMC Article
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

Prognosis

López SI, Ciocca M, Oleastro M, Cuarterolo ML, Rocca A, de Dávila MT, Roy A, Fernández MC, Nievas E, Bosaleh A, Torgerson TR, Ruiz JA
J Pediatr Gastroenterol Nutr 2011 Dec;53(6):690-3. doi: 10.1097/MPG.0b013e3182250651. PMID: 21629128
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
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

Invernizzi P, Alessio MG, Smyk DS, Lleo A, Sonzogni A, Fabris L, Candusso M, Bogdanos DP, Iorio R, Torre G
BMC Gastroenterol 2012 Jul 20;12:92. doi: 10.1186/1471-230X-12-92. PMID: 22816667Free PMC Article
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
Obermayer-Straub P, Manns MP
Mol Biol Rep 1996;23(3-4):235-42. doi: 10.1007/BF00351174. PMID: 9112234

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