Details of Disease

General Information of Disease (ID: DISOX03Q)

Disease Name Autoimmune hepatitis
Synonyms AIH; autoimmune chronic hepatitis; autoimmune hepatitis with centrilobular necrosis; chronic autoimmune hepatitis; autoimmune chronic active hepatitis; autoimmune liver disease; autoimmune hepatitis
Disease Class DB96: Autoimmune liver disease
Definition Hepatitis caused by autoantibodies. Drugs, infections, and toxins may trigger the production of the autoantibodies against the liver parenchyma.
Disease Hierarchy
DISEMG4F: Autoimmune disorder of exocrine system
DISLAIH9: Autoimmune disorder of gastrointestinal tract
DISTWCN9: Autoimmune disorder of endocrine system
DISXXX35: Hepatitis
DISOX03Q: Autoimmune hepatitis
ICD Code
ICD-11
ICD-11: DB96.0
ICD-10
ICD-10: K75.4
Expand ICD-11
'DB96.0
Expand ICD-10
'K75.4
Disease Identifiers
MONDO ID
MONDO_0016264
UMLS CUI
C4721555
MedGen ID
1666753
Orphanet ID
2137
SNOMED CT ID
408335007

Drug-Interaction Atlas (DIA) of This Disease

Drug-Interaction Atlas (DIA)
This Disease is Treated as An Indication in 1 Clinical Trial Drug(s)
Drug Name Drug ID Highest Status Drug Type REF
SPIF DMZDE82 Phase 1 NA [1]
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This Disease is Treated as An Indication in 1 Preclinical Drug(s)
Drug Name Drug ID Highest Status Drug Type REF
mRNA-6981 DMG6NCM Preclinical Vaccine [2]
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Molecular Interaction Atlas (MIA) of This Disease

Molecular Interaction Atlas (MIA)
This Disease Is Related to 10 DTT Molecule(s)
Gene Name DTT ID Evidence Level Mode of Inheritance REF
ASPH TT2KHP7 Strong Biomarker [3]
CXCR1 TTMWT8Z Strong Biomarker [4]
CYP1A2 TTS1DTU Strong Biomarker [5]
CYP2D6 TTVG215 Strong Biomarker [6]
FGL2 TTYBS89 Strong Biomarker [7]
GEM TTAZF9M Strong Biomarker [8]
GPR39 TTTPCNU Strong Biomarker [9]
RORC TTGV6LY Strong Biomarker [10]
TBX21 TTNF9PH Strong Genetic Variation [11]
TEC TT1ZV49 Strong Biomarker [12]
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⏷ Show the Full List of 10 DTT(s)
This Disease Is Related to 2 DME Molecule(s)
Gene Name DME ID Evidence Level Mode of Inheritance REF
GPT DER5HFI Strong Biomarker [13]
TPMT DEFQ8VO Strong Biomarker [14]
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This Disease Is Related to 33 DOT Molecule(s)
Gene Name DOT ID Evidence Level Mode of Inheritance REF
PDC OT1UUVYY Limited Genetic Variation [15]
ACTA1 OTOVGLPG Strong Altered Expression [16]
AMELX OTIN26MM Strong Biomarker [17]
C4A OTXMOYXU Strong Genetic Variation [18]
C6orf120 OTPBRZZR Strong Biomarker [19]
CARD10 OT2RPM4I Strong Genetic Variation [20]
CYP2D7 OTYJTL3S Strong Biomarker [21]
DLAT OT9LBJVN Strong Biomarker [22]
ENAM OTK8PU0T Strong Biomarker [23]
FANCC OTTIDM3P Strong Biomarker [24]
FBL OTRODIE5 Strong Biomarker [25]
FCRL3 OTIFXFWL Strong Genetic Variation [26]
FH OTEQWU6Q Strong Altered Expression [27]
FTCD OTX9HZT5 Strong Biomarker [28]
HLA-DPB1 OTW8JHU2 Strong Genetic Variation [29]
HLA-DRB3 OT5PM9N7 Strong Genetic Variation [30]
HLA-DRB4 OTNXIHQU Strong Genetic Variation [31]
IL17C OTKXIVNQ Strong Biomarker [32]
IL17RE OTMFTNLS Strong Biomarker [32]
ISG20 OTCWRJJW Strong Biomarker [16]
KIR3DL1 OTPOSXFX Strong Biomarker [8]
KLF14 OT8BXLBS Strong Biomarker [33]
MLKL OTDSLC81 Strong Altered Expression [34]
NUDT15 OTX8SZOT Strong Biomarker [14]
OGT OT1Z1ZXE Strong Altered Expression [35]
PGAM5 OT6Q5M0C Strong Altered Expression [36]
PPIG OTZ8BTTM Strong Biomarker [37]
PTPN22 OTDCNTC3 Strong Genetic Variation [38]
RNPC3 OTW5MKC1 Strong Biomarker [39]
RPS20 OTI8052R Strong Biomarker [40]
SEPSECS OTP0FHOV Strong Biomarker [41]
STXBP2 OTX8GUC4 Strong Genetic Variation [42]
JAM3 OTX0F9QL Definitive Altered Expression [43]
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⏷ Show the Full List of 33 DOT(s)

References

1 Clinical pipeline report, company report or official report of the Pharmaceutical Research and Manufacturers of America (PhRMA)
2 Clinical pipeline report, company report or official report of Moderna
3 Pediatric and adult forms of type I autoimmune hepatitis in Argentina: evidence for differential genetic predisposition.Hepatology. 1999 Dec;30(6):1374-80. doi: 10.1002/hep.510300611.
4 Usefulness of chemokine C-C receptor 7(-) /programmed cell death-1(+) follicular helper T cell subset frequencies in the diagnosis of autoimmune hepatitis.Hepatol Res. 2019 Sep;49(9):1026-1033. doi: 10.1111/hepr.13356. Epub 2019 Jun 3.
5 Autoimmune polyendocrine syndrome type 1: an extensive longitudinal study in Sardinian patients.J Clin Endocrinol Metab. 2012 Apr;97(4):1114-24. doi: 10.1210/jc.2011-2461. Epub 2012 Feb 16.
6 Chemical induced inflammation of the liver breaks tolerance and results in autoimmune hepatitis in Balb/c mice.Immunol Lett. 2020 Feb;218:44-50. doi: 10.1016/j.imlet.2019.11.010. Epub 2019 Nov 30.
7 Soluble Fgl2 restricts autoimmune hepatitis progression via suppressing Tc17 and conventional CD8+ T cell function.J Gene Med. 2018 Jul;20(7-8):e3023. doi: 10.1002/jgm.3023. Epub 2018 Jun 19.
8 The early onset of type 1 autoimmune hepatitis has a strong genetic influence: role of HLA and KIR genes.Genes Immun. 2016 Apr;17(3):187-92. doi: 10.1038/gene.2016.7. Epub 2016 Feb 18.
9 G Protein-Coupled Receptor 39 Agonist Improves Concanavalin A-Induced Hepatitis in Mice.Biol Pharm Bull. 2019 Aug 1;42(8):1415-1418. doi: 10.1248/bpb.b18-00982. Epub 2019 Jun 4.
10 The impaired immune regulation of autoimmune hepatitis is linked to a defective galectin-9/tim-3 pathway.Hepatology. 2012 Aug;56(2):677-86. doi: 10.1002/hep.25682. Epub 2012 Jul 6.
11 Influence of TBX21 T-1993C variant on autoimmune hepatitis development by Yin-Yang 1 binding.World J Gastroenterol. 2017 Dec 28;23(48):8500-8511. doi: 10.3748/wjg.v23.i48.8500.
12 Medullary thymic epithelial cell depletion leads to autoimmune hepatitis.J Clin Invest. 2013 Aug;123(8):3510-24. doi: 10.1172/JCI65414. Epub 2013 Jul 15.
13 Long-term clinical outcomes in patients with autoimmune hepatitis according to treatment response in Asian country.Liver Int. 2019 May;39(5):985-994. doi: 10.1111/liv.14082. Epub 2019 Mar 25.
14 NUDT15 Polymorphism Confer Increased Susceptibility to Thiopurine-Induced Leukopenia in Patients With Autoimmune Hepatitis and Related Cirrhosis.Front Pharmacol. 2019 Apr 9;10:346. doi: 10.3389/fphar.2019.00346. eCollection 2019.
15 Autoimmune regulator AIRE: evidence for genetic differences between autoimmune hepatitis and hepatitis as part of the autoimmune polyglandular syndrome type 1.Hepatology. 2001 May;33(5):1047-52. doi: 10.1053/jhep.2001.24031.
16 Foxp3?Treg Cells Are Associated with Pathological Process of Autoimmune Hepatitis by Activating Methylation Modification in Autoimmune Hepatitis Patients.Med Sci Monit. 2019 Aug 18;25:6204-6212. doi: 10.12659/MSM.915408.
17 Tryptophan-kynurenine profile in pediatric autoimmune hepatitis.Immunol Res. 2019 Feb;67(1):39-47. doi: 10.1007/s12026-019-9068-1.
18 Early-onset autoimmune hepatitis is associated with a C4A gene deletion.Gastroenterology. 1993 May;104(5):1478-84. doi: 10.1016/0016-5085(93)90359-k.
19 Deletion of the C6orf120 gene with unknown function ameliorates autoimmune hepatitis induced by concanavalin A.Cell Immunol. 2018 Sep;331:9-15. doi: 10.1016/j.cellimm.2018.04.017. Epub 2018 May 2.
20 Genome-wide association study identifies variants associated with autoimmune hepatitis type 1.Gastroenterology. 2014 Aug;147(2):443-52.e5. doi: 10.1053/j.gastro.2014.04.022. Epub 2014 Apr 23.
21 Overlapping but distinct specificities of anti-liver-kidney microsome antibodies in autoimmune hepatitis type II and hepatitis C revealed by recombinant native CYP2D6 and novel peptide epitopes.Clin Exp Immunol. 1999 Nov;118(2):290-7. doi: 10.1046/j.1365-2249.1999.01027.x.
22 Suitability of the simplified autoimmune hepatitis score for the diagnosis of autoimmune hepatitis in a German cohort.United European Gastroenterol J. 2018 Mar;6(2):247-254. doi: 10.1177/2050640617711632. Epub 2017 May 18.
23 Diagnosis and Management of Pediatric Autoimmune Liver Disease: ESPGHAN Hepatology Committee Position Statement.J Pediatr Gastroenterol Nutr. 2018 Feb;66(2):345-360. doi: 10.1097/MPG.0000000000001801.
24 (18)F-FAC PET Selectively Images Liver-Infiltrating CD4 and CD8 T Cells in a Mouse Model of Autoimmune Hepatitis.J Nucl Med. 2018 Oct;59(10):1616-1623. doi: 10.2967/jnumed.118.210328. Epub 2018 Apr 26.
25 Noninvasive indices for monitoring disease course in Chinese patients with autoimmune hepatitis.Clin Chim Acta. 2018 Nov;486:135-141. doi: 10.1016/j.cca.2018.07.030. Epub 2018 Jul 20.
26 Lack of association between FCRL3 and FcgammaRII polymorphisms in Japanese type 1 autoimmune hepatitis.Clin Immunol. 2007 Mar;122(3):338-42. doi: 10.1016/j.clim.2006.08.012. Epub 2006 Oct 3.
27 Fumarate hydratase-specific T cell response in Chinese patients with autoimmune hepatitis.Clin Res Hepatol Gastroenterol. 2018 Sep;42(4):339-346. doi: 10.1016/j.clinre.2017.12.003. Epub 2018 Mar 31.
28 The induction of autoimmune hepatitis in the human leucocyte antigen-DR4 non-obese diabetic mice autoimmune hepatitis mouse model.Clin Exp Immunol. 2016 Nov;186(2):164-176. doi: 10.1111/cei.12843. Epub 2016 Aug 23.
29 A cis-eQTL of HLA-DPB1 Affects Susceptibility to Type 1 Autoimmune Hepatitis.Sci Rep. 2018 Aug 9;8(1):11924. doi: 10.1038/s41598-018-30406-9.
30 Genetic heterogeneity in susceptibility to autoimmune hepatitis types 1 and 2.Am J Gastroenterol. 1999 Jul;94(7):1906-13. doi: 10.1111/j.1572-0241.1999.01229.x.
31 Genetic analysis of the HLA region of Japanese patients with type 1 autoimmune hepatitis.J Hepatol. 2005 Apr;42(4):578-84. doi: 10.1016/j.jhep.2004.12.019. Epub 2005 Jan 21.
32 IL-17C/IL-17RE Augments T Cell Function in Autoimmune Hepatitis.J Immunol. 2017 Jan 15;198(2):669-680. doi: 10.4049/jimmunol.1600977. Epub 2016 Dec 12.
33 Overexpression of KLF14 protects against immune-mediated hepatic injury in mice.Lab Invest. 2019 Jan;99(1):37-47. doi: 10.1038/s41374-018-0134-4. Epub 2018 Sep 25.
34 The pseudokinase MLKL mediates programmed hepatocellular necrosis independently of RIPK3 during hepatitis.J Clin Invest. 2016 Nov 1;126(11):4346-4360. doi: 10.1172/JCI87545. Epub 2016 Oct 17.
35 Deficient O-GlcNAc Glycosylation Impairs Regulatory T Cell Differentiation and Notch Signaling in Autoimmune Hepatitis.Front Immunol. 2018 Oct 9;9:2089. doi: 10.3389/fimmu.2018.02089. eCollection 2018.
36 PGAM5-mediated programmed necrosis of hepatocytes drives acute liver injury.Gut. 2017 Apr;66(4):716-723. doi: 10.1136/gutjnl-2015-311247. Epub 2016 Aug 26.
37 Autoantibodies against CYP-2C19: A Novel Serum Marker in Pediatric De Novo Autoimmune Hepatitis?.Biomed Res Int. 2017;2017:3563278. doi: 10.1155/2017/3563278. Epub 2017 Nov 27.
38 Association of STAT4, TGF1, SH2B3 and PTPN22 polymorphisms with autoimmune hepatitis.Exp Mol Pathol. 2018 Dec;105(3):279-284. doi: 10.1016/j.yexmp.2018.10.001. Epub 2018 Oct 3.
39 Anti-tRNP(ser)sec/SLA/LP autoantibodies. Comparative study using in-house ELISA with a recombinant 48.8 kDa protein, immunoblot, and analysis of immunoprecipitated RNAs.Liver Int. 2005 Apr;25(2):410-9. doi: 10.1111/j.1478-3231.2005.01079.x.
40 Novel autoimmune hepatitis-specific autoantigens identified using protein microarray technology.J Proteome Res. 2010 Jan;9(1):30-9. doi: 10.1021/pr900131e.
41 Permanent immunosuppression in SLA/LP-positive autoimmune hepatitis is required although overall response and survival are similar.Liver Int. 2020 Feb;40(2):368-376. doi: 10.1111/liv.14280. Epub 2019 Nov 1.
42 Novel Patient with Late-Onset Familial Hemophagocytic Lymphohistiocytosis with STXBP2 Mutations Presenting with Autoimmune Hepatitis, Neurological Manifestations and Infections Associated with Hypogammaglobulinemia.J Clin Immunol. 2015 Jan;35(1):22-5. doi: 10.1007/s10875-014-0119-z. Epub 2014 Dec 10.
43 Junctional adhesion molecules JAM-B and JAM-C promote autoimmune-mediated liver fibrosis in mice.J Autoimmun. 2018 Jul;91:83-96. doi: 10.1016/j.jaut.2018.05.001. Epub 2018 May 9.