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Variant Autoimmune Hepatitis

MedGen UID:
1779326
•Concept ID:
C5448070
•
Disease or Syndrome
Synonyms: Autoimmune Hepatitis, Variant; Variant AIH

Definition

A form of autoimmune hepatitis (AIH) that has features of overlapping cholestatic autoimmune liver diseases including primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC), or autoimmune cholangitis (AIC). [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVVariant Autoimmune Hepatitis

Professional guidelines

PubMed

Epidemiology, clinical features and management of autoimmune hepatitis in Switzerland: a retrospective and prospective cohort study.
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
Individualizing Care: Management Beyond Medical Therapy.
Cristoferi L, Nardi A, Invernizzi P, Mells G, Carbone M
Clin Liver Dis 2018 Aug;22(3):545-561. doi: 10.1016/j.cld.2018.03.006. PMID: 30259852
Oral lichen planus and lichenoid reactions: etiopathogenesis, diagnosis, management and malignant transformation.
Ismail SB, Kumar SK, Zain RB
J Oral Sci 2007 Jun;49(2):89-106. doi: 10.2334/josnusd.49.89. PMID: 17634721

Recent clinical studies

Etiology

Incidence and predictors of hepatocellular carcinoma in patients with autoimmune hepatitis.
Colapietro F, Maisonneuve P, Lytvyak E, Beuers U, Verdonk RC, van der Meer AJ, van Hoek B, Kuiken SD, Brouwer JT, Muratori P, Aghemo A, Carella F, van den Berg AP, Zachou K, Dalekos GN, Di Zeo-Sánchez DE, Robles M, Andrade RJ, Montano-Loza AJ, van den Brand FF, Slooter CD, Macedo G, Liberal R, de Boer YS, Lleo A; Dutch AIH Study Group; International Autoimmune Hepatitis Group
J Hepatol 2024 Jan;80(1):53-61. Epub 2023 Oct 5 doi: 10.1016/j.jhep.2023.09.010. PMID: 37802188
Insights into new-onset autoimmune diseases after COVID-19 vaccination.
Guo M, Liu X, Chen X, Li Q
Autoimmun Rev 2023 Jul;22(7):103340. Epub 2023 Apr 17 doi: 10.1016/j.autrev.2023.103340. PMID: 37075917Free PMC Article
Monogenic early-onset lymphoproliferation and autoimmunity: Natural history of STAT3 gain-of-function syndrome.
Leiding JW, Vogel TP, Santarlas VGJ, Mhaskar R, Smith MR, Carisey A, Vargas-Hernández A, Silva-Carmona M, Heeg M, Rensing-Ehl A, Neven B, Hadjadj J, Hambleton S, Ronan Leahy T, Meesilpavikai K, Cunningham-Rundles C, Dutmer CM, Sharapova SO, Taskinen M, Chua I, Hague R, Klemann C, Kostyuchenko L, Morio T, Thatayatikom A, Ozen A, Scherbina A, Bauer CS, Flanagan SE, Gambineri E, Giovannini-Chami L, Heimall J, Sullivan KE, Allenspach E, Romberg N, Deane SG, Prince BT, Rose MJ, Bohnsack J, Mousallem T, Jesudas R, Santos Vilela MMD, O'Sullivan M, Pachlopnik Schmid J, Průhová Š, Klocperk A, Rees M, Su H, Bahna S, Baris S, Bartnikas LM, Chang Berger A, Briggs TA, Brothers S, Bundy V, Chan AY, Chandrakasan S, Christiansen M, Cole T, Cook MC, Desai MM, Fischer U, Fulcher DA, Gallo S, Gauthier A, Gennery AR, Gonçalo Marques J, Gottrand F, Grimbacher B, Grunebaum E, Haapaniemi E, Hämäläinen S, Heiskanen K, Heiskanen-Kosma T, Hoffman HM, Gonzalez-Granado LI, Guerrerio AL, Kainulainen L, Kumar A, Lawrence MG, Levin C, Martelius T, Neth O, Olbrich P, Palma A, Patel NC, Pozos T, Preece K, Lugo Reyes SO, Russell MA, Schejter Y, Seroogy C, Sinclair J, Skevofilax E, Suan D, Suez D, Szabolcs P, Velasco H, Warnatz K, Walkovich K, Worth A; STAT3 GOF Working Group members, Seppänen MRJ, Torgerson TR, Sogkas G, Ehl S, Tangye SG, Cooper MA, Milner JD, Forbes Satter LR
J Allergy Clin Immunol 2023 Apr;151(4):1081-1095. Epub 2022 Oct 11 doi: 10.1016/j.jaci.2022.09.002. PMID: 36228738Free PMC Article
Lichen planus pigmentosus and its variants: review and update.
Robles-Méndez JC, Rizo-Frías P, Herz-Ruelas ME, Pandya AG, Ocampo Candiani J
Int J Dermatol 2018 May;57(5):505-514. Epub 2017 Oct 26 doi: 10.1111/ijd.13806. PMID: 29076159
Drug-induced liver injury.
Katarey D, Verma S
Clin Med (Lond) 2016 Dec;16(Suppl 6):s104-s109. doi: 10.7861/clinmedicine.16-6-s104. PMID: 27956449Free PMC Article

Diagnosis

Monogenic early-onset lymphoproliferation and autoimmunity: Natural history of STAT3 gain-of-function syndrome.
Leiding JW, Vogel TP, Santarlas VGJ, Mhaskar R, Smith MR, Carisey A, Vargas-Hernández A, Silva-Carmona M, Heeg M, Rensing-Ehl A, Neven B, Hadjadj J, Hambleton S, Ronan Leahy T, Meesilpavikai K, Cunningham-Rundles C, Dutmer CM, Sharapova SO, Taskinen M, Chua I, Hague R, Klemann C, Kostyuchenko L, Morio T, Thatayatikom A, Ozen A, Scherbina A, Bauer CS, Flanagan SE, Gambineri E, Giovannini-Chami L, Heimall J, Sullivan KE, Allenspach E, Romberg N, Deane SG, Prince BT, Rose MJ, Bohnsack J, Mousallem T, Jesudas R, Santos Vilela MMD, O'Sullivan M, Pachlopnik Schmid J, Průhová Š, Klocperk A, Rees M, Su H, Bahna S, Baris S, Bartnikas LM, Chang Berger A, Briggs TA, Brothers S, Bundy V, Chan AY, Chandrakasan S, Christiansen M, Cole T, Cook MC, Desai MM, Fischer U, Fulcher DA, Gallo S, Gauthier A, Gennery AR, Gonçalo Marques J, Gottrand F, Grimbacher B, Grunebaum E, Haapaniemi E, Hämäläinen S, Heiskanen K, Heiskanen-Kosma T, Hoffman HM, Gonzalez-Granado LI, Guerrerio AL, Kainulainen L, Kumar A, Lawrence MG, Levin C, Martelius T, Neth O, Olbrich P, Palma A, Patel NC, Pozos T, Preece K, Lugo Reyes SO, Russell MA, Schejter Y, Seroogy C, Sinclair J, Skevofilax E, Suan D, Suez D, Szabolcs P, Velasco H, Warnatz K, Walkovich K, Worth A; STAT3 GOF Working Group members, Seppänen MRJ, Torgerson TR, Sogkas G, Ehl S, Tangye SG, Cooper MA, Milner JD, Forbes Satter LR
J Allergy Clin Immunol 2023 Apr;151(4):1081-1095. Epub 2022 Oct 11 doi: 10.1016/j.jaci.2022.09.002. PMID: 36228738Free PMC Article
Lichen planus pigmentosus and its variants: review and update.
Robles-Méndez JC, Rizo-Frías P, Herz-Ruelas ME, Pandya AG, Ocampo Candiani J
Int J Dermatol 2018 May;57(5):505-514. Epub 2017 Oct 26 doi: 10.1111/ijd.13806. PMID: 29076159
Drug-induced liver injury.
Katarey D, Verma S
Clin Med (Lond) 2016 Dec;16(Suppl 6):s104-s109. doi: 10.7861/clinmedicine.16-6-s104. PMID: 27956449Free PMC Article
Autoimmune hepatitis.
Heneghan MA, Yeoman AD, Verma S, Smith AD, Longhi MS
Lancet 2013 Oct 26;382(9902):1433-44. Epub 2013 Jun 14 doi: 10.1016/S0140-6736(12)62163-1. PMID: 23768844
Oral lichen planus and lichenoid reactions: etiopathogenesis, diagnosis, management and malignant transformation.
Ismail SB, Kumar SK, Zain RB
J Oral Sci 2007 Jun;49(2):89-106. doi: 10.2334/josnusd.49.89. PMID: 17634721

Therapy

Causal effects of gut microbiome on autoimmune liver disease: a two-sample Mendelian randomization study.
Fu Y, Li J, Zhu Y, Chen C, Liu J, Gu S, Zheng Y, Li Y
BMC Med Genomics 2023 Oct 3;16(1):232. doi: 10.1186/s12920-023-01670-0. PMID: 37789337Free PMC Article
Insights into new-onset autoimmune diseases after COVID-19 vaccination.
Guo M, Liu X, Chen X, Li Q
Autoimmun Rev 2023 Jul;22(7):103340. Epub 2023 Apr 17 doi: 10.1016/j.autrev.2023.103340. PMID: 37075917Free PMC Article
Pathogenesis of Autoimmune Hepatitis-Cellular and Molecular Mechanisms.
Sirbe C, Simu G, Szabo I, Grama A, Pop TL
Int J Mol Sci 2021 Dec 17;22(24) doi: 10.3390/ijms222413578. PMID: 34948375Free PMC Article
Overlap Syndromes.
Chazouillères O
Dig Dis 2015;33 Suppl 2:181-7. Epub 2015 Dec 7 doi: 10.1159/000440831. PMID: 26641819
Oral lichen planus and lichenoid reactions: etiopathogenesis, diagnosis, management and malignant transformation.
Ismail SB, Kumar SK, Zain RB
J Oral Sci 2007 Jun;49(2):89-106. doi: 10.2334/josnusd.49.89. PMID: 17634721

Prognosis

Incidence and predictors of hepatocellular carcinoma in patients with autoimmune hepatitis.
Colapietro F, Maisonneuve P, Lytvyak E, Beuers U, Verdonk RC, van der Meer AJ, van Hoek B, Kuiken SD, Brouwer JT, Muratori P, Aghemo A, Carella F, van den Berg AP, Zachou K, Dalekos GN, Di Zeo-Sánchez DE, Robles M, Andrade RJ, Montano-Loza AJ, van den Brand FF, Slooter CD, Macedo G, Liberal R, de Boer YS, Lleo A; Dutch AIH Study Group; International Autoimmune Hepatitis Group
J Hepatol 2024 Jan;80(1):53-61. Epub 2023 Oct 5 doi: 10.1016/j.jhep.2023.09.010. PMID: 37802188
Insights into new-onset autoimmune diseases after COVID-19 vaccination.
Guo M, Liu X, Chen X, Li Q
Autoimmun Rev 2023 Jul;22(7):103340. Epub 2023 Apr 17 doi: 10.1016/j.autrev.2023.103340. PMID: 37075917Free PMC Article
Lichen planus pigmentosus and its variants: review and update.
Robles-Méndez JC, Rizo-Frías P, Herz-Ruelas ME, Pandya AG, Ocampo Candiani J
Int J Dermatol 2018 May;57(5):505-514. Epub 2017 Oct 26 doi: 10.1111/ijd.13806. PMID: 29076159
Chronic Granulomatous Disease.
Rawat A, Bhattad S, Singh S
Indian J Pediatr 2016 Apr;83(4):345-53. Epub 2016 Feb 11 doi: 10.1007/s12098-016-2040-3. PMID: 26865172
Autoimmune hepatitis.
Heneghan MA, Yeoman AD, Verma S, Smith AD, Longhi MS
Lancet 2013 Oct 26;382(9902):1433-44. Epub 2013 Jun 14 doi: 10.1016/S0140-6736(12)62163-1. PMID: 23768844

Clinical prediction guides

Genetic association and causal relationship between multiple modifiable risk factors and autoimmune liver disease: a two-sample mendelian randomization study.
Gao W, Peng C, Wang Z, Li Y, Liu M
J Transl Med 2024 May 4;22(1):425. doi: 10.1186/s12967-024-05247-y. PMID: 38704596Free PMC Article
Incidence and predictors of hepatocellular carcinoma in patients with autoimmune hepatitis.
Colapietro F, Maisonneuve P, Lytvyak E, Beuers U, Verdonk RC, van der Meer AJ, van Hoek B, Kuiken SD, Brouwer JT, Muratori P, Aghemo A, Carella F, van den Berg AP, Zachou K, Dalekos GN, Di Zeo-Sánchez DE, Robles M, Andrade RJ, Montano-Loza AJ, van den Brand FF, Slooter CD, Macedo G, Liberal R, de Boer YS, Lleo A; Dutch AIH Study Group; International Autoimmune Hepatitis Group
J Hepatol 2024 Jan;80(1):53-61. Epub 2023 Oct 5 doi: 10.1016/j.jhep.2023.09.010. PMID: 37802188
Monogenic early-onset lymphoproliferation and autoimmunity: Natural history of STAT3 gain-of-function syndrome.
Leiding JW, Vogel TP, Santarlas VGJ, Mhaskar R, Smith MR, Carisey A, Vargas-Hernández A, Silva-Carmona M, Heeg M, Rensing-Ehl A, Neven B, Hadjadj J, Hambleton S, Ronan Leahy T, Meesilpavikai K, Cunningham-Rundles C, Dutmer CM, Sharapova SO, Taskinen M, Chua I, Hague R, Klemann C, Kostyuchenko L, Morio T, Thatayatikom A, Ozen A, Scherbina A, Bauer CS, Flanagan SE, Gambineri E, Giovannini-Chami L, Heimall J, Sullivan KE, Allenspach E, Romberg N, Deane SG, Prince BT, Rose MJ, Bohnsack J, Mousallem T, Jesudas R, Santos Vilela MMD, O'Sullivan M, Pachlopnik Schmid J, Průhová Š, Klocperk A, Rees M, Su H, Bahna S, Baris S, Bartnikas LM, Chang Berger A, Briggs TA, Brothers S, Bundy V, Chan AY, Chandrakasan S, Christiansen M, Cole T, Cook MC, Desai MM, Fischer U, Fulcher DA, Gallo S, Gauthier A, Gennery AR, Gonçalo Marques J, Gottrand F, Grimbacher B, Grunebaum E, Haapaniemi E, Hämäläinen S, Heiskanen K, Heiskanen-Kosma T, Hoffman HM, Gonzalez-Granado LI, Guerrerio AL, Kainulainen L, Kumar A, Lawrence MG, Levin C, Martelius T, Neth O, Olbrich P, Palma A, Patel NC, Pozos T, Preece K, Lugo Reyes SO, Russell MA, Schejter Y, Seroogy C, Sinclair J, Skevofilax E, Suan D, Suez D, Szabolcs P, Velasco H, Warnatz K, Walkovich K, Worth A; STAT3 GOF Working Group members, Seppänen MRJ, Torgerson TR, Sogkas G, Ehl S, Tangye SG, Cooper MA, Milner JD, Forbes Satter LR
J Allergy Clin Immunol 2023 Apr;151(4):1081-1095. Epub 2022 Oct 11 doi: 10.1016/j.jaci.2022.09.002. PMID: 36228738Free PMC Article
NAFLD and autoimmune hepatitis: Do not judge a book by its cover.
Dalekos GN, Gatselis NK, Zachou K, Koukoulis GK
Eur J Intern Med 2020 May;75:1-9. Epub 2020 Feb 9 doi: 10.1016/j.ejim.2020.02.001. PMID: 32051092
Variant syndromes of primary biliary cholangitis.
Schulz L, Sebode M, Weidemann SA, Lohse AW
Best Pract Res Clin Gastroenterol 2018 Jun-Aug;34-35:55-61. Epub 2018 Jun 14 doi: 10.1016/j.bpg.2018.06.003. PMID: 30343711

Recent systematic reviews

Skin Manifestations Associated with Autoimmune Liver Diseases: a Systematic Review.
Terziroli Beretta-Piccoli B, Invernizzi P, Gershwin ME, Mainetti C
Clin Rev Allergy Immunol 2017 Dec;53(3):394-412. doi: 10.1007/s12016-017-8649-9. PMID: 28993983
Systematic review: management options for primary sclerosing cholangitis and its variant forms - IgG4-associated cholangitis and overlap with autoimmune hepatitis.
Culver EL, Chapman RW
Aliment Pharmacol Ther 2011 Jun;33(12):1273-91. Epub 2011 Apr 18 doi: 10.1111/j.1365-2036.2011.04658.x. PMID: 21501198

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