AIH
MCID: ATM011
MIFTS: 60

Autoimmune Hepatitis (AIH) malady

Rare diseases, Gastrointestinal diseases, Liver diseases, Immune diseases, Infectious diseases categories
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Summaries for Autoimmune Hepatitis

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Sources:
8Disease Ontology, 43NIH Rare Diseases, 65Wikipedia, 33MalaCards
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NIH Rare Diseases:43 Autoimmune hepatitis is a disease in which the body’s immune system attacks liver cells. this immune response causes inflammation of the liver, also called hepatitis. the disease can be quite serious and, if not treated, gets worse over time, leading to cirrhosis of the liver and/or liver failure. autoimmune hepatitis sometimes occurs in relatives of people with autoimmune diseases, suggesting a genetic cause. this disease is most common in young girls and women. last updated: 6/8/2011

MalaCards: Autoimmune Hepatitis, also known as autoimmune hepatitis with centrilobular necrosis, is related to hepatitis and primary biliary cirrhosis. An important gene associated with Autoimmune Hepatitis is SEPSECS (Sep (O-phosphoserine) tRNA:Sec (selenocysteine) tRNA synthase), and among its related pathways are One carbon pool by folate and Metabolism. The compounds perhexiline and thiopurine have been mentioned in the context of this disorder. Affiliated tissues include liver, liver and t cells, and related mouse phenotypes are liver/biliary system and normal.

Disease Ontology:8 An autoimmune disease of gastrointestinal tract that results in inflammation located in liver caused by the body's immune system attacking the liver cells.

Wikipedia:65 Autoimmune Hepatitis is a disease of the liver that occurs when the body\'s immune system attacks cells... more...

Aliases & Classifications for Autoimmune Hepatitis

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Sources:
8Disease Ontology, 43NIH Rare Diseases, 10DISEASES, 49Orphanet, 31LifeMap Discovery™, 45Novoseek, 62UMLS, 58SNOMED-CT, 40NCIt, 27ICD9CM, 35MeSH, 26ICD10 via Orphanet, 63UMLS via Orphanet, 59SNOMED-CT via Orphanet, 25ICD10
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Classifications:

Orphanet: 49 
Rare hepatic diseases


Characteristics (Orphanet epidemiological data):

49
autoimmune hepatitis:
Inheritance: Sporadic; Prevalence: 1-9/1000000; Age of onset: Adulthood


Aliases & Descriptions:

autoimmune hepatitis 8 43 10 49 31
autoimmune hepatitis with centrilobular necrosis 8
autoimmune chronic active hepatitis 8
chronic autoimmune hepatitis 49
autoimmune chronic hepatitis 43
hepatitis, autoimmune 62
hepatitis autoimmune 45
aih 43


External Ids:

Disease Ontology8 DOID:2048
NCIt40 C27029
ICD9CM27 571.42
MeSH35 D019693
ICD10 via Orphanet26 K75.4
UMLS via Orphanet63 C0241910
SNOMED-CT via Orphanet59 408335007
ICD1025 K75.4

Related Diseases for Autoimmune Hepatitis

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Sources:
17GeneCards, 18GeneDecks
See all sources

Diseases in the Hepatitis C family:

autoimmune hepatitis Hepatitis B
Hepatitis D Hepatitis a
Hepatitis E Hepatitis
Viral Hepatitis Hepatitis C Virus

Diseases related to Autoimmune Hepatitis via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 150)
idRelated DiseaseScoreTop Affiliating Genes
1hepatitis32.1ARG1, SEPSECS
2primary biliary cirrhosis31.3AIRE, SEPSECS, CTLA4
3thyroiditis30.9CTLA4
4lupus erythematosus30.8C4A
5systemic lupus erythematosus30.8C4A
6rheumatoid arthritis30.5TNFRSF6B, TPMT, FCRL3, CTLA4
7autoimmune thyroiditis30.3CTLA4, AIRE
8thymoma30.3AIRE, CTLA4
9vasculitis30.2CTLA4, TPMT
10graves' disease30.2CTLA4, FCRL3, AIRE
11vitiligo30.0CTLA4, AIRE
12myasthenia gravis30.0CTLA4, AIRE
13multiple sclerosis30.0CYP2D6, TPMT, CTLA4
14addison's disease30.0AIRE, CYP2D6, CTLA4
15diabetes mellitus29.9CTLA4, AIRE
16hepatitis a10.7
17cholangitis10.7
18sclerosing cholangitis10.6
19hepatitis c10.6
20primary sclerosing cholangitis10.6
21hepatocellular carcinoma10.5
22liver disease10.5
23celiac disease10.4
24arthritis10.4
25connective tissue disease10.4
26ulcerative colitis10.3
27mixed connective tissue disease10.3
28crohn's disease10.3
29hepatitis b10.3
30adult-onset still's disease10.3
31hemolytic anemia10.3
32purpura10.3
33polymyositis10.3
34acute liver failure10.3
35autoimmune hemolytic anemia10.3
36viral hepatitis10.3
37hepatitis c virus10.3
38bernard-soulier syndrome10.2
39polyarteritis nodosa10.2
40visceral leishmaniasis10.2
41inflammatory bowel disease10.2
42leishmaniasis10.2
43liver cirrhosis10.2
44thrombocytopenia10.2
45evans' syndrome10.1
46membranous glomerulonephritis10.1
47pyoderma gangrenosum10.1
48autoimmune polyendocrine syndrome10.1
49glomerulonephritis10.1
50hypothyroidism10.1

Graphical network of the top 20 diseases related to Autoimmune Hepatitis:



Diseases related to autoimmune hepatitis

Symptoms for Autoimmune Hepatitis

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Drugs & Therapeutics for Autoimmune Hepatitis

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Sources:
42NIH Clinical Center, 6ClinicalTrials, 31LifeMap Discovery™, 62UMLS, 41NDF-RT
See all sources

Drug clinical trials:

Search ClinicalTrials for Autoimmune Hepatitis

Search NIH Clinical Center for Autoimmune Hepatitis

Cell-based therapeutics:


LifeMap Discovery
The database of embryonic development, stem cell research and regenerative medicine
Read about Autoimmune Hepatitis cell therapies at LifeMap Discovery.

Genetic Tests for Autoimmune Hepatitis

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Anatomical Context for Autoimmune Hepatitis

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Sources:
31LifeMap Discovery™, 33MalaCards, 14FMA
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MalaCards organs/tissues related to Autoimmune Hepatitis:

33
Liver, T cells, Thyroid, Bone marrow, Bone, Kidney, Pancreas, Smooth muscle, B cells, Testes, Lymph node, Heart, Spleen, Myeloid, Skin

FMA organs/tissues related to Autoimmune Hepatitis:

14
Liver

LifeMap Discovery
The database of embryonic development, stem cell research and regenerative medicine

Cells/anatomical compartments in embryo or adult related to Autoimmune Hepatitis:
id TissueAnatomical CompartmentCell Relevance
1 BloodHematopoietic Bone MarrowHematopoietic Stem Cells Potential therapeutic candidate
2 Umbilical CordWharton's JellyMesenchymal Stem Cells Potential therapeutic candidate

Animal Models for Autoimmune Hepatitis or affiliated genes

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Sources:
37MGI
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MGI Mouse Phenotypes related to Autoimmune Hepatitis:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053708.7ARG1, CTLA4, ASIP, AIRE
2MP:00028737.9AIRE, PTPRCAP, ASIP, CTLA4, ARG1
3MP:00053977.8ARG1, CTLA4, ASIP, TPMT, PTPRCAP, AIRE

Publications for Autoimmune Hepatitis

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Sources:
52PubMed
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Articles related to Autoimmune Hepatitis:

(show top 50)    (show all 774)
idTitleAuthorsYear
1
Review article: the prevention and reversal of hepatic fibrosis in autoimmune hepatitis. (24387318)
2014
2
Hashimoto's thyroiditis concomitant with sequential autoimmune hepatitis, chorea and polyserositis: a new entity of autoimmune polyendocrine syndrome? (23318858)
2013
3
Autoimmune hepatitis type-2 and Epstein-Barr virus infection in a toddler: art of facts or an artifact? (23293207)
2013
4
Infliximab as a rescue treatment in difficult-to-treat autoimmune hepatitis. (23178709)
2013
5
SPRi-Based Strategy to Identify Specific Biomarkers in Systemic Lupus Erythematosus, Rheumatoid Arthritis and Autoimmune Hepatitis. (24376828)
2013
6
Autoimmune hepatitis on the rise. (24295874)
2013
7
Clinical features of autoimmune hepatitis and comparison of two diagnostic criteria in Korea: a nationwide, multicenter study. (23033899)
2013
8
Budesonide versus prednisone with azathioprine for the treatment of autoimmune hepatitis in children and adolescents. (23810723)
2013
9
Anti-nucleosome autoantibodies as markers for autoimmune hepatitis and their correlation with disease activity. (23607768)
2013
10
Drug choices in autoimmune hepatitis: part A--Steroids. (23061711)
2012
11
Liver transplantation for autoimmune hepatitis in Peru: outcomes and recurrence. (22345339)
2012
12
The CYP2D6 animal model: how to induce autoimmune hepatitis in mice. (22331063)
2012
13
Role of thiopurine metabolite measurement in the management of autoimmune hepatitis. (22589202)
2012
14
Clinical features and long term outcome of 102 treated autoimmune hepatitis patients. (22509185)
2012
15
Histopathology of de novo autoimmune hepatitis. (22378542)
2012
16
Acquired haemophilia A in a woman with autoimmune hepatitis and systemic lupus erythematosus; review of literature. (22027758)
2012
17
Hepatitis surface antigen clearance in a woman with liver cirrhosis and concomitant autoimmune hepatitis under immunosuppressive therapy. (23181542)
2012
18
Diagnosis and treatment of autoimmune hepatitis. (22179843)
2012
19
Autoimmune hepatitis in children: an overview of the disease focusing on current therapies. (22495399)
2012
20
Association of TBX21 promoter polymorphisms with type 1 autoimmune hepatitis in a Chinese population. (20977921)
2011
21
Optimizing management in autoimmune hepatitis with liver failure at initial presentation. (21547124)
2011
22
Advanced histology and impaired liver regeneration are associated with disease severity in acute-onset autoimmune hepatitis. (21401703)
2011
23
The benefit of animal models for autoimmune hepatitis. (22117631)
2011
24
Mycophenolate mofetil as a rescue therapy for autoimmune hepatitis patients who are not responsive to standard therapy. (21780898)
2011
25
Requirements and challenges of a preclinical autoimmune hepatitis mouse model. (21894011)
2011
26
Performance of the international classifications criteria for autoimmune hepatitis diagnosis in Mexican patients. (21875376)
2011
27
Seven cases of autoimmune hepatitis that developed after drug-induced liver injury. (21725992)
2011
28
Acquired haemophilia A in a woman with autoimmune hepatitis and systemic lupus erythematosus: review of literature. (21885954)
2011
29
Risk factors associated with relapse of type 1 autoimmune hepatitis in Japan. (21711423)
2011
30
Autoimmune hepatitis type 2 following anti-papillomavirus vaccination in a 11-year-old girl. (21596082)
2011
31
Clinical expression of autoimmune hepatitis in a nine-year-old girl with visceral leishmaniasis. (21866471)
2011
32
Another cause of autoimmune hepatitis. (20578154)
2010
33
Corticosteroid-induced mania in a patient with autoimmune hepatitis. (20220360)
2010
34
Recurrence of autoimmune disease, primary sclerosing cholangitis, primary biliary cirrhosis, and autoimmune hepatitis after liver transplantation. (19876939)
2009
35
Clinical features of 107 autoimmune hepatitis patients and 30 of them with AIH-primary biliary cirrhosis overlap syndrome]. (18510851)
2008
36
Effects of serum aspartate aminotransferase levels in patients with autoimmune hepatitis influence disease course and outcome. (18840547)
2008
37
A functional Fas promoter polymorphism is associated with a severe phenotype in type 1 autoimmune hepatitis characterized by early development of cirrhosis. (17493146)
2007
38
Selenocysteine, soluble liver antigen/liver-pancreas, and autoimmune hepatitis. (17596869)
2007
39
Utility of thiopurine methyltransferase genotyping and phenotyping, and measurement of azathioprine metabolites in the management of patients with autoimmune hepatitis. (16876902)
2006
40
Scleroderma-like lesions in the course of autoimmune hepatitis. Case report]. (16334539)
2005
41
Use of etanercept in treatment of pyoderma gangrenosum in a patient with autoimmune hepatitis. (16428159)
2005
42
Secondary IgA nephropathy presenting as nephrotic syndrome with glomerular crescentic changes and acute renal failure in a patient with autoimmune hepatitis. (15151269)
2004
43
A case of autoimmune hepatitis and autoimmune hemolytic anemia following hepatitis A infection. (14655068)
2003
44
HLA genotyping in type-I autoimmune hepatitis in Western India. (14719586)
2003
45
Autoimmune hepatitis and overlap syndrome: therapy]. (12233265)
2002
46
Anti-cytokeratin antibodies in sera of the patients with autoimmune hepatitis. (11529922)
2001
47
Autoimmune regulator AIRE: evidence for genetic differences between autoimmune hepatitis and hepatitis as part of the autoimmune polyglandular syndrome type 1. (11343230)
2001
48
Autoimmune cholangitis with features of autoimmune hepatitis: successful treatment with immunosuppressive agents and ursodeoxycholic acid. (10719755)
2000
49
The liver in collagen diseases: pathologic study of 160 cases with particular reference to hepatic arteritis, primary biliary cirrhosis, autoimmune hepatitis and nodular regenerative hyperplasia of the liver. (11092254)
2000
50
Apoptosis and hepatic cell proliferation in autoimmune hepatitis and chronic viral hepatitis C in children: analysis of APO-1/Fas (CD95), bcl-2 and Ki67 expression]. (10910654)
1999

Variations for Autoimmune Hepatitis

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Expression for genes affiliated with Autoimmune Hepatitis

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Sources:
2BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Autoimmune Hepatitis

Search GEO for disease gene expression data for Autoimmune Hepatitis.

Pathways for genes affiliated with Autoimmune Hepatitis

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Sources:
50PathCards, 30KEGG, 38NCBI BioSystems Database, 51PharmGKB, 60Thomson Reuters, 55Reactome
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Pathways related to Autoimmune Hepatitis according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
One Carbon Metabolism38
dTMP de novo biosynthesis (mitochondrial)38
tetrahydrofolate salvage from 5,10-methenyltetrahydrofolate38
Trans-sulfuration and one carbon metabolism38
methionine salvage38
Methionine metabolism60
9.8FTCD, TPMT
2
Show member pathways
8.4ARG1, FTCD, TPMT, TNFRSF6B, CYP2D6

Compounds for genes affiliated with Autoimmune Hepatitis

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Sources:
45Novoseek, 11DrugBank, 51PharmGKB, 29IUPHAR, 24HMDB, 61Tocris Bioscience
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Compounds related to Autoimmune Hepatitis according to GeneCards/GeneDecks:

(show all 12)
idCompoundScoreTop Affiliating Genes
1perhexiline45 1111.0CYP2D6, TPMT
2thiopurine4510.0TPMT, CYP2D6
3halothane45 51 29 1113.0SEPSECS, CYP2D6
4Pyridoxal 5'-phosphate2410.0FTCD, SEPSECS
5uridine diphosphate4510.0TPMT, CYP2D6
6bortezomib45 51 1111.9CTLA4, CYP2D6
7mycophenolate mofetil45 51 1111.8CTLA4, SEPSECS, TPMT
8xanthine45 2410.7TPMT, CYP2D6
9isoleucine459.5CYP2D6, ARG1
10cyclosporin a45 29 6111.3CTLA4, SEPSECS, TPMT
11tyrosine458.7AIRE, CYP2D6, PTPRCAP, TPMT, CTLA4
12steroid458.7C4A, CTLA4, SEPSECS, TPMT, CYP2D6

GO Terms for genes affiliated with Autoimmune Hepatitis

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Products for genes affiliated with Autoimmune Hepatitis

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  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Autoimmune Hepatitis

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4CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
52PubMed
53QIAGEN
59SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet