MCID: ATM113
MIFTS: 33

Autoimmune Cholangitis

Categories: Gastrointestinal diseases, Immune diseases, Liver diseases
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Aliases & Classifications for Autoimmune Cholangitis

MalaCards integrated aliases for Autoimmune Cholangitis:

Name: Autoimmune Cholangitis 11 14
Autoimmune Cholangiopathy 11

Classifications:



External Ids:

Disease Ontology 11 DOID:0080742

Summaries for Autoimmune Cholangitis

Disease Ontology: 11 An autoimmune hepatitis that is characterized by primary biliary cirrhosis clinical, biochemical, and histologic characteristics with antinuclear antibody positive sera.

MalaCards based summary: Autoimmune Cholangitis, also known as autoimmune cholangiopathy, is related to igg4-related disease and cholangitis. An important gene associated with Autoimmune Cholangitis is DLAT (Dihydrolipoamide S-Acetyltransferase), and among its related pathways/superpathways are Innate Immune System and PAK Pathway. Affiliated tissues include liver, t cells and salivary gland, and related phenotypes are immune system and renal/urinary system

Related Diseases for Autoimmune Cholangitis

Diseases in the Cholangitis family:

Autoimmune Cholangitis Acute Cholangitis
Chronic Cholangitis

Diseases related to Autoimmune Cholangitis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 428)
# Related Disease Score Top Affiliating Genes
1 igg4-related disease 30.6 CD4 CCR6 CA2
2 cholangitis 30.6 SP100 IL17A DLAT CA2 ABCB4
3 autoimmune pancreatitis 30.5 CD4 CCR6 CA2
4 hashimoto thyroiditis 30.2 CD8A CD4 CCR6
5 celiac disease 1 30.2 IL17A CD8A CD4 CCR6
6 anal squamous cell carcinoma 30.1 CD8A CD4
7 panniculitis 30.1 CD8A CD4 CCR6
8 chronic fatigue syndrome 30.1 CD8A CD4 CCR6
9 drug-induced hepatitis 29.9 SEPSECS DNAL1 CD4
10 anemia, autoimmune hemolytic 29.9 RAG1 CD8A CD4 CCR6
11 colitis 29.8 IL23A IL17A CD4 CCR6
12 viral infectious disease 29.6 SP100 IL17A CD4 CCR6
13 chronic cholangitis 29.5 SP100 NUP210 DLAT ABCB4
14 sclerosing cholangitis 28.8 SP100 SEPSECS NUP210 IL17A DNAL1 DLAT
15 cholangitis, primary sclerosing 28.8 SP100 SEPSECS NUP210 IL17A DNAL1 DLAT
16 primary biliary cholangitis 28.4 SP100 SEPSECS PDHX NUP210 IL17A IL12RB2
17 liver cirrhosis 10.5
18 autoimmune hepatitis 10.5
19 cholestasis 10.4
20 autoimmune disease 10.4
21 hilar lung neoplasm 10.4 CD8A CD4
22 oral tuberculosis 10.3 CD8A CD4
23 early yaws 10.3 CD8A CD4
24 ventilation pneumonitis 10.3 CD8A CD4
25 diffuse infiltrative lymphocytosis syndrome 10.3 CD8A CD4
26 wissler-fanconi syndrome 10.3 CD8A CD4
27 autoimmune lymphoproliferative syndrome, type iia 10.3 CD8A CD4
28 encephalitozoonosis 10.3 CD8A CD4
29 type 1 diabetes mellitus 23 10.3 CD8A CD4
30 cork-handlers' disease 10.3 CD8A CD4
31 follicular mucinosis 10.3 CD8A CD4
32 jejunoileitis 10.3 CD8A CCR6
33 suppurative lymphadenitis 10.3 CD8A CD4
34 bird fancier's lung 10.3 CD8A CD4
35 parapsoriasis 10.3 CD8A CD4
36 primary syphilis 10.3 CD8A CD4
37 granulomatous angiitis 10.3 CD8A CD4
38 primary cutaneous gamma-delta t-cell lymphoma 10.3 CD8A CD4
39 thymic dysplasia 10.3 CD8A CD4
40 splenic abscess 10.3 CD8A CD4
41 mooren's ulcer 10.3 CD8A CD4
42 herpes simplex virus keratitis 10.3 CD8A CD4
43 granulomatous dermatitis 10.3 CD8A CD4
44 anti-basement membrane glomerulonephritis 10.3 CD4 CCR6
45 acute retinal necrosis syndrome 10.3 CD8A CD4
46 granulomatous hepatitis 10.3 CD8A CD4
47 orbital cellulitis 10.3 CD8A CD4
48 acute orbital inflammation 10.3 CD8A CD4
49 viral exanthem 10.3 CD8A CD4
50 tertiary syphilis 10.3 CD8A CD4

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



Diseases related to Autoimmune Cholangitis

Symptoms & Phenotypes for Autoimmune Cholangitis

GenomeRNAi Phenotypes related to Autoimmune Cholangitis according to GeneCards Suite gene sharing:

25
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 no effect GR00402-S-1 10.1 ABCB4 CA2 CCR6 CD4 CD8A DBT
2 no effect GR00402-S-2 10.1 ABCB4 CA2 CCR6 CD4 DBT DLAT

MGI Mouse Phenotypes related to Autoimmune Cholangitis:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 immune system MP:0005387 10.03 ABCB4 CA2 CCR6 CD4 CD8A DNAL1
2 renal/urinary system MP:0005367 9.97 CA2 CD8A DBT DNAL1 IL12RB2 IL17A
3 digestive/alimentary MP:0005381 9.97 ABCB4 CA2 CD4 DNAL1 IL12RB2 IL17A
4 hematopoietic system MP:0005397 9.73 ABCB4 CCR6 CD4 CD8A DNAL1 IL12RB2
5 respiratory system MP:0005388 9.7 CA2 DBT DNAL1 IL17A RAG1 SEPSECS
6 mortality/aging MP:0010768 9.4 CA2 CD4 CD8A DBT DLAT DNAL1

Drugs & Therapeutics for Autoimmune Cholangitis

Search Clinical Trials, NIH Clinical Center for Autoimmune Cholangitis

Genetic Tests for Autoimmune Cholangitis

Anatomical Context for Autoimmune Cholangitis

Organs/tissues related to Autoimmune Cholangitis:

MalaCards : Liver, T Cells, Salivary Gland, Lymph Node, Nk Cells, B Cells, Monocytes

Publications for Autoimmune Cholangitis

Articles related to Autoimmune Cholangitis:

(show top 50) (show all 278)
# Title Authors PMID Year
1
Treatment with a JAK1/2 inhibitor ameliorates murine autoimmune cholangitis induced by IFN overexpression. 62
36042351 2022
2
Dual B-cell targeting therapy ameliorates autoimmune cholangitis. 62
36029718 2022
3
Immunoglobulin G4 associated autoimmune cholangitis and pancreatitis following the administration of nivolumab: A case report. 62
36051149 2022
4
Migratory Panniculitis with Autoimmune Cholangitis and Pancreatitis (IgG4- Related Disease): A Rare Presentation. 62
36386064 2022
5
Interplay between Mast Cells and Regulatory T Cells in Immune-Mediated Cholangiopathies. 62
35682552 2022
6
Warm autoimmune haemolytic anaemia seen in association with primary sclerosing cholangitis in the setting of Klebsiella pneumoniae bacteraemia. 62
35606025 2022
7
Isolated IgG4 Autoimmune Cholangitis in the Absence of Pancreatitis. 62
35371842 2022
8
Clinical aspects and prognosis of patients with inflammatory bowel disease associated with autoimmune liver diseases. 62
34023469 2022
9
Gender-specific development of experimental autoimmune cholangitis induced by double-stranded RNA. 62
34953211 2022
10
The Proinflammatory Cytokines IL-18, IL-21, and IFN-γ Differentially Regulate Liver Inflammation and Anti-Mitochondrial Antibody Level in a Murine Model of Primary Biliary Cholangitis. 62
35300072 2022
11
A phase II study of buparlisib in relapsed or refractory thymomas. 62
36330484 2022
12
New insights on the role of human leukocyte antigen complex in primary biliary cholangitis. 62
36119102 2022
13
Gut microbiota contributes to sexual dimorphism in murine autoimmune cholangitis. 62
34047390 2021
14
Enoxacin Up-Regulates MicroRNA Biogenesis and Down-Regulates Cytotoxic CD8 T-Cell Function in Autoimmune Cholangitis. 62
33462854 2021
15
Interleukin-30 Suppresses Not Only CD4+ T Cells but Also Regulatory T Cells in Murine Primary Biliary Cholangitis. 62
34440235 2021
16
IL-17A/F enable cholangiocytes to restrict T cell-driven experimental cholangitis by upregulating PD-L1 expression. 62
33197512 2021
17
Nanoparticle-mediated targeting of autoantigen peptide to cross-presenting liver sinusoidal endothelial cells protects from CD8 T-cell-driven autoimmune cholangitis. 62
33346377 2021
18
Differences in Clinical Features and Diagnostic Strategies Between IgG4-Related Autoimmune Cholangitis and Cholangiocarcinoma. 62
33816216 2021
19
Interleukin 23 Produced by Hepatic Monocyte-Derived Macrophages Is Essential for the Development of Murine Primary Biliary Cholangitis. 62
34484224 2021
20
Glycomic analysis of antibody indicates distinctive glycosylation profile in patients with autoimmune cholangitis. 62
32546343 2020
21
Treatment of Overlap Syndromes in Autoimmune Liver Disease: A Systematic Review and Meta-Analysis. 62
32414025 2020
22
Liver-resident NK cells suppress autoimmune cholangitis and limit the proliferation of CD4+ T cells. 62
30874628 2020
23
Invasive Hepatobiliary Trichosporon asahii Infection in a Child With Autoimmune Cholangitis. 62
31107957 2019
24
HLA-DRB1 GENE POLYMORPHISMS IN PEDIATRIC PATIENTS WITH TYPE 1 AUTOIMMUNE HEPATITIS AND TYPE 1 AUTOIMMUNE HEPATITIS OVERLAP SYNDROME WITH AUTOIMMUNE CHOLANGITIS. 62
31460577 2019
25
[Is autoimmune cholangitis-anti-mitochondrial antibody-negative primary biliary cholangitis still an independent disease?] 62
31177668 2019
26
Mig chemokine in primary biliary cirrhosis. 62
31173052 2019
27
Sarcoidosis of the Intra- and Extrahepatic Bile Ducts with Concomitant Cholangitis in a Patient with Ulcerative Colitis. 62
31097931 2019
28
Gal-3 Deficiency Suppresses Novosphyngobium aromaticivorans Inflammasome Activation and IL-17 Driven Autoimmune Cholangitis in Mice. 62
31231399 2019
29
Gut microbiota translocation promotes autoimmune cholangitis. 62
30340822 2018
30
Endogenous IL-10 maintains immune tolerance but IL-10 gene transfer exacerbates autoimmune cholangitis. 62
30274824 2018
31
Type 1 and Type 2 Autoimmune Pancreatitis: Distinctive Clinical and Pathological Features, But Are There Any Differences at Magnetic Resonance? Experience From a Referral Center. 62
30141780 2018
32
Mesenchymal stem cells alleviate experimental autoimmune cholangitis through immunosuppression and cytoprotective function mediated by galectin-9. 62
30223894 2018
33
Proteomic analysis reveals distinctive protein profiles involved in CD8+ T cell-mediated murine autoimmune cholangitis. 62
29375127 2018
34
The interplay of type I and type II interferons in murine autoimmune cholangitis as a basis for sex-biased autoimmunity. 62
28921595 2018
35
[Primary biliary cholangitis. Part 2. State of the art, diagnosis, associated diseases, treatment and prognosis]. 62
29791424 2018
36
The Critical Role of Chemokine (C-C Motif) Receptor 2-Positive Monocytes in Autoimmune Cholangitis. 62
30158929 2018
37
The CXC Chemokine Receptor 3 Inhibits Autoimmune Cholangitis via CD8+ T Cells but Promotes Colitis via CD4+ T Cells. 62
29868034 2018
38
A Novel Pkhd1 Mutation Interacts with the Nonobese Diabetic Genetic Background To Cause Autoimmune Cholangitis. 62
29158418 2018
39
A Mouse Model of Autoimmune Cholangitis via Syngeneic Bile Duct Protein Immunization. 62
29127360 2017
40
[Autoimmune hepatitis: Immunological diagnosis]. 62
28919271 2017
41
[Primary biliary cholangitis. Part 1. State of the art, epidemiology, physiopathology and clinical manifestations]. 62
29459807 2017
42
The clinical extremes of autoimmune cholangitis. 62
28376623 2017
43
Chemokine receptor CXCR3 deficiency exacerbates murine autoimmune cholangitis by promoting pathogenic CD8+ T cell activation. 62
28129932 2017
44
Diagnosis and Treatment of Autoimmune Liver Diseases in a Tertiary Referral Center in Cuba. 62
29158853 2017
45
Outcome and Genetic Factors in IgG4-Associated Autoimmune Pancreatitis and Cholangitis: A Single Center Experience. 62
28348580 2017
46
Galectin-3 regulates inflammasome activation in cholestatic liver injury. 62
27630169 2016
47
The diagnosis of antimitochondrial antibody-negative primary biliary cholangitis. 62
27567165 2016
48
Prevalence of IgG-4-associated cholangiopathy based on serum IgG-4 levels in patients with primary sclerosing cholangitis and its relationship with inflammatory bowel disease. 62
27852547 2016
49
Chronic expression of interferon-gamma leads to murine autoimmune cholangitis with a female predominance. 62
27178326 2016
50
Dual Roles of IFN-γ and IL-4 in the Natural History of Murine Autoimmune Cholangitis: IL-30 and Implications for Precision Medicine. 62
27721424 2016

Variations for Autoimmune Cholangitis

Expression for Autoimmune Cholangitis

Search GEO for disease gene expression data for Autoimmune Cholangitis.

Pathways for Autoimmune Cholangitis

Pathways related to Autoimmune Cholangitis according to GeneCards Suite gene sharing:

(show all 13)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.73 SP100 RAG1 NUP210 IL23A IL17A IL12RB2
2
Show member pathways
13.02 TGFBR2 IL23A IL17A IL12RB2 CD4 CCR6
3
Show member pathways
12.95 SP100 RAG1 NUP210 IL23A IL17A IL12RB2
4
Show member pathways
11.73 IL12RB2 CD8A CD4
5
Show member pathways
11.63 IL17A IL12RB2 CD4
6
Show member pathways
11.58 TGFBR2 IL23A IL17A
7
Show member pathways
11.54 IL23A IL17A IL12RB2 CD8A CD4
8 11.45 CD4 CD8A IL23A
9
Show member pathways
11.42 TGFBR2 IL23A IL17A CD4
10 11.38 IL23A IL17A CD4
11
Show member pathways
11.01 PDHX DLAT DBT
12 11 IL23A IL17A IL12RB2 CD8A CD4 CCR6
13 10.67 IL12RB2 CD4

GO Terms for Autoimmune Cholangitis

Cellular components related to Autoimmune Cholangitis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 external side of plasma membrane GO:0009897 9.73 TGFBR2 IL17A IL12RB2 CD8A CD4 CCR6
2 mitochondrial pyruvate dehydrogenase complex GO:0005967 9.56 PDHX DLAT
3 pyruvate dehydrogenase complex GO:0045254 9.46 PDHX DLAT
4 oxidoreductase complex GO:1990204 8.8 PDHX DLAT DBT

Biological processes related to Autoimmune Cholangitis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 immune response GO:0006955 9.36 RAG1 IL23A IL17A CD8A CD4 CCR6
2 acetyl-CoA biosynthetic process from pyruvate GO:0006086 9.26 PDHX DLAT

Molecular functions related to Autoimmune Cholangitis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 pyruvate dehydrogenase (NAD+) activity GO:0034604 8.92 PDHX DLAT

Sources for Autoimmune Cholangitis

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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