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Autoimmune hepatitis type 1

MedGen UID:
928833
•Concept ID:
C4303164
•
Disease or Syndrome
Synonyms: AIH type 1; autoimmune hepatitis type 1; Autoimmune Hepatitis, Type 1; Type 1 AIH; type 1 AIH; Type 1 Autoimmune Hepatitis
SNOMED CT: Autoimmune hepatitis type 1 (721711009)
 
Monarch Initiative: MONDO:0005543
Orphanet: ORPHA563576

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 antinuclear antibodies (ANA), anti-smooth muscle antibodies (anti-SMA), and/or anti-soluble liver antigen/liver pancreas antigen antibodies (anti-SLA/LP). The disease predominantly develops at a post-pubertal age and most commonly takes a chronic course, although acute or acute severe presentation may also be observed. Typical concurrent autoimmune diseases are autoimmune thyroiditis and rheumatic diseases. [from ORDO]

Term Hierarchy

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

Professional guidelines

PubMed

Aminotransferases During Treatment Predict Long-Term Survival in Patients With Autoimmune Hepatitis Type 1: A Landmark Analysis.
Biewenga M, Verhelst X, Baven-Pronk M, Putter H, van den Berg A, Colle I, Schouten J, Sermon F, Van Steenkiste C, van Vlierberghe H, van der Meer A, van Hoek B; Dutch Autoimmune Hepatitis Study Group
Clin Gastroenterol Hepatol 2022 Aug;20(8):1776-1783.e4. Epub 2021 May 20 doi: 10.1016/j.cgh.2021.05.024. PMID: 34022454
Consequences of treatment withdrawal in type 1 autoimmune hepatitis.
Montano-Loza AJ, Carpenter HA, Czaja AJ
Liver Int 2007 May;27(4):507-15. doi: 10.1111/j.1478-3231.2007.01444.x. PMID: 17403191
Drug therapy in the management of type 1 autoimmune hepatitis.
Czaja AJ
Drugs 1999 Jan;57(1):49-68. doi: 10.2165/00003495-199957010-00005. PMID: 9951951

Recent clinical studies

Etiology

HLA, gut microbiome and hepatic autoimmunity.
Terziroli Beretta-Piccoli B, Mieli-Vergani G, Vergani D
Front Immunol 2022;13:980768. Epub 2022 Aug 18 doi: 10.3389/fimmu.2022.980768. PMID: 36059527Free PMC Article
Expression of HLA and Autoimmune Pathway Genes in Liver Biopsies of Young Subjects With Autoimmune Hepatitis Type 1.
Shin E, Schwarz KB, Jones-Brando LV, Florea LD, Sabunciyan S, Wood LD, Yolken RH
J Pediatr Gastroenterol Nutr 2022 Sep 1;75(3):269-275. Epub 2022 Jun 27 doi: 10.1097/MPG.0000000000003538. PMID: 35759748Free PMC Article
Genome-wide meta-analysis identifies susceptibility loci for autoimmune hepatitis type 1.
Li Y, Sun Y, Liu Y, Wang B, Li J, Wang H, Zhang H, Wang X, Han X, Lin Q, Zhou Y, Hu L, Song Y, Bao J, Gong L, Sun M, Yuan X, Zhang X, Lian M, Xiao X, Miao Q, Wang Q, Li KK, Du S, Ma A, Li Y, Xu J, Tang S, Shi J, Xu Y, Yang L, Zhang J, Huang Z, Zhou L, Cui Y, Seldin MF, Gershwin ME, Yan H, Zou Z, Zuo X, Tang R, Ma X; Chinese AIH Consortium
Hepatology 2022 Sep;76(3):564-575. Epub 2022 Jun 3 doi: 10.1002/hep.32417. PMID: 35184318
Development and validation of a prognostic score for long-term transplant-free survival in autoimmune hepatitis type 1.
Biewenga M, Verhelst XPDMJ, Baven-Pronk MAMC, Putter H, van den Berg AP, van Nieuwkerk KCMJ, van Buuren HR, Bouma G, de Boer YS, Simoen C, Colle I, Schouten J, Sermon F, van Steenkiste C, van Vlierberghe H, van der Meer AJ, Nevens F, van Hoek B; Dutch Autoimmune Hepatitis Study Group
United European Gastroenterol J 2021 Jul;9(6):662-671. Epub 2021 Jun 24 doi: 10.1002/ueg2.12112. PMID: 34165262Free PMC Article
Presentation and Outcomes of Autoimmune Hepatitis Type 1 and Type 2 in Children: A Single-center Study.
Roepe IG, Vierling JM, Goss JA, Miloh T
J Pediatr Gastroenterol Nutr 2021 Jan 1;72(1):101-107. doi: 10.1097/MPG.0000000000002892. PMID: 32796427

Diagnosis

Serum Biomarkers for Autoimmune Hepatitis Type 1: the Case for CD48 and a Review of the Literature.
Hu M, You Z, Li Y, Huang B, Cui N, Wang R, Wei Y, Li B, Liang J, Liu Q, Li Y, Wang H, Qian Q, Zhang J, Chen R, Lyu Z, Chen Y, Xiao X, Lian M, Tang R, Miao Q, Wang Q, Ma X
Clin Rev Allergy Immunol 2022 Dec;63(3):342-356. Epub 2022 Jun 3 doi: 10.1007/s12016-022-08935-z. PMID: 35657576
Serum Immunoglobulin G Levels Predict Biochemical and Histological Remission of Autoimmune Hepatitis Type 1: A Single-Center Experience and Literature Review.
Li Y, Yan L, Wang R, Wang Q, You Z, Li B, Zhang J, Huang B, Chen Y, Li Y, Lian M, Tang R, Qiu D, Gershwin ME, Xiao X, Miao Q, Ma X
Clin Rev Allergy Immunol 2022 Apr;62(2):292-300. Epub 2021 Jan 29 doi: 10.1007/s12016-021-08833-w. PMID: 33512642
Presentation and Outcomes of Autoimmune Hepatitis Type 1 and Type 2 in Children: A Single-center Study.
Roepe IG, Vierling JM, Goss JA, Miloh T
J Pediatr Gastroenterol Nutr 2021 Jan 1;72(1):101-107. doi: 10.1097/MPG.0000000000002892. PMID: 32796427
2020 international consensus on ANCA testing beyond systemic vasculitis.
Moiseev S, Cohen Tervaert JW, Arimura Y, Bogdanos DP, Csernok E, Damoiseaux J, Ferrante M, Flores-Suárez LF, Fritzler MJ, Invernizzi P, Jayne D, Jennette JC, Little MA, McAdoo SP, Novikov P, Pusey CD, Radice A, Salama AD, Savige JA, Segelmark M, Shoenfeld Y, Sinico RA, Sousa MJ, Specks U, Terrier B, Tzioufas AG, Vermeire S, Zhao MH, Bossuyt X
Autoimmun Rev 2020 Sep;19(9):102618. Epub 2020 Jul 12 doi: 10.1016/j.autrev.2020.102618. PMID: 32663621
Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy from the pediatric perspective.
Capalbo D, Improda N, Esposito A, De Martino L, Barbieri F, Betterle C, Pignata C, Salerno M
J Endocrinol Invest 2013 Nov;36(10):903-12. Epub 2013 May 30 doi: 10.3275/8999. PMID: 23723078

Therapy

Anti-TNFα Treatment in Children and Adolescents With Combined Inflammatory Bowel Disease and Autoimmune Liver Disease.
Nedelkopoulou N, Vadamalayan B, Vergani D, Mieli-Vergani G
J Pediatr Gastroenterol Nutr 2018 Jan;66(1):100-105. doi: 10.1097/MPG.0000000000001759. PMID: 28953529
Characteristics and outcome of autoimmune liver disease in Asian children.
Lee WS, Lum SH, Lim CB, Chong SY, Khoh KM, Ng RT, Teo KM, Boey CC, Pailoor J
Hepatol Int 2015 Apr;9(2):292-302. Epub 2014 Aug 6 doi: 10.1007/s12072-014-9558-0. PMID: 25788179
Risk factors for hepatocellular carcinoma in Japanese patients with autoimmune hepatitis type 1.
Hino-Arinaga T, Ide T, Kuromatsu R, Miyajima I, Ogata K, Kuwahara R, Hisamochi A, Torimura T, Sata M; Autoimmune Hepatitis Study Group
J Gastroenterol 2012 May;47(5):569-76. Epub 2011 Dec 21 doi: 10.1007/s00535-011-0519-2. PMID: 22187167
Antibodies to filamentous actin (F-actin) in type 1 autoimmune hepatitis.
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
Autoimmune hepatitis type 1: safety and efficacy of prolonged medical therapy.
Seela S, Sheela H, Boyer JL
Liver Int 2005 Aug;25(4):734-9. doi: 10.1111/j.1478-3231.2005.01141.x. PMID: 15998423

Prognosis

HLA, gut microbiome and hepatic autoimmunity.
Terziroli Beretta-Piccoli B, Mieli-Vergani G, Vergani D
Front Immunol 2022;13:980768. Epub 2022 Aug 18 doi: 10.3389/fimmu.2022.980768. PMID: 36059527Free PMC Article
Serum Biomarkers for Autoimmune Hepatitis Type 1: the Case for CD48 and a Review of the Literature.
Hu M, You Z, Li Y, Huang B, Cui N, Wang R, Wei Y, Li B, Liang J, Liu Q, Li Y, Wang H, Qian Q, Zhang J, Chen R, Lyu Z, Chen Y, Xiao X, Lian M, Tang R, Miao Q, Wang Q, Ma X
Clin Rev Allergy Immunol 2022 Dec;63(3):342-356. Epub 2022 Jun 3 doi: 10.1007/s12016-022-08935-z. PMID: 35657576
Serum Immunoglobulin G Levels Predict Biochemical and Histological Remission of Autoimmune Hepatitis Type 1: A Single-Center Experience and Literature Review.
Li Y, Yan L, Wang R, Wang Q, You Z, Li B, Zhang J, Huang B, Chen Y, Li Y, Lian M, Tang R, Qiu D, Gershwin ME, Xiao X, Miao Q, Ma X
Clin Rev Allergy Immunol 2022 Apr;62(2):292-300. Epub 2021 Jan 29 doi: 10.1007/s12016-021-08833-w. PMID: 33512642
Development and validation of a prognostic score for long-term transplant-free survival in autoimmune hepatitis type 1.
Biewenga M, Verhelst XPDMJ, Baven-Pronk MAMC, Putter H, van den Berg AP, van Nieuwkerk KCMJ, van Buuren HR, Bouma G, de Boer YS, Simoen C, Colle I, Schouten J, Sermon F, van Steenkiste C, van Vlierberghe H, van der Meer AJ, Nevens F, van Hoek B; Dutch Autoimmune Hepatitis Study Group
United European Gastroenterol J 2021 Jul;9(6):662-671. Epub 2021 Jun 24 doi: 10.1002/ueg2.12112. PMID: 34165262Free PMC Article
Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy from the pediatric perspective.
Capalbo D, Improda N, Esposito A, De Martino L, Barbieri F, Betterle C, Pignata C, Salerno M
J Endocrinol Invest 2013 Nov;36(10):903-12. Epub 2013 May 30 doi: 10.3275/8999. PMID: 23723078

Clinical prediction guides

Serum Biomarkers for Autoimmune Hepatitis Type 1: the Case for CD48 and a Review of the Literature.
Hu M, You Z, Li Y, Huang B, Cui N, Wang R, Wei Y, Li B, Liang J, Liu Q, Li Y, Wang H, Qian Q, Zhang J, Chen R, Lyu Z, Chen Y, Xiao X, Lian M, Tang R, Miao Q, Wang Q, Ma X
Clin Rev Allergy Immunol 2022 Dec;63(3):342-356. Epub 2022 Jun 3 doi: 10.1007/s12016-022-08935-z. PMID: 35657576
Aminotransferases During Treatment Predict Long-Term Survival in Patients With Autoimmune Hepatitis Type 1: A Landmark Analysis.
Biewenga M, Verhelst X, Baven-Pronk M, Putter H, van den Berg A, Colle I, Schouten J, Sermon F, Van Steenkiste C, van Vlierberghe H, van der Meer A, van Hoek B; Dutch Autoimmune Hepatitis Study Group
Clin Gastroenterol Hepatol 2022 Aug;20(8):1776-1783.e4. Epub 2021 May 20 doi: 10.1016/j.cgh.2021.05.024. PMID: 34022454
Serum Immunoglobulin G Levels Predict Biochemical and Histological Remission of Autoimmune Hepatitis Type 1: A Single-Center Experience and Literature Review.
Li Y, Yan L, Wang R, Wang Q, You Z, Li B, Zhang J, Huang B, Chen Y, Li Y, Lian M, Tang R, Qiu D, Gershwin ME, Xiao X, Miao Q, Ma X
Clin Rev Allergy Immunol 2022 Apr;62(2):292-300. Epub 2021 Jan 29 doi: 10.1007/s12016-021-08833-w. PMID: 33512642
Development and validation of a prognostic score for long-term transplant-free survival in autoimmune hepatitis type 1.
Biewenga M, Verhelst XPDMJ, Baven-Pronk MAMC, Putter H, van den Berg AP, van Nieuwkerk KCMJ, van Buuren HR, Bouma G, de Boer YS, Simoen C, Colle I, Schouten J, Sermon F, van Steenkiste C, van Vlierberghe H, van der Meer AJ, Nevens F, van Hoek B; Dutch Autoimmune Hepatitis Study Group
United European Gastroenterol J 2021 Jul;9(6):662-671. Epub 2021 Jun 24 doi: 10.1002/ueg2.12112. PMID: 34165262Free PMC Article
HLA-DRB1*03:01 and HLA-DRB1*04:01 modify the presentation and outcome in autoimmune hepatitis type-1.
van Gerven NM, de Boer YS, Zwiers A, Verwer BJ, Drenth JP, van Hoek B, van Erpecum KJ, Beuers U, van Buuren HR, den Ouden JW, Verdonk RC, Koek GH, Brouwer JT, Guichelaar MM, Vrolijk JM, Coenraad MJ, Kraal G, Mulder CJ, van Nieuwkerk CM, Bloemena E, Verspaget HW, Kumar V, Zhernakova A, Wijmenga C, Franke L, Bouma G; Dutch Autoimmune Hepatitis Study Group
Genes Immun 2015 Jun;16(4):247-52. Epub 2015 Jan 22 doi: 10.1038/gene.2014.82. PMID: 25611558

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