AIH
MCID: ATM011
MIFTS: 63

Autoimmune Hepatitis (AIH)

Categories: Cancer diseases, Gastrointestinal diseases, Genetic diseases, Immune diseases, Infectious diseases, Liver diseases, Rare diseases, Skin diseases

Aliases & Classifications for Autoimmune Hepatitis

MalaCards integrated aliases for Autoimmune Hepatitis:

Name: Autoimmune Hepatitis 12 74 52 58 36 29 15 37 17 32
Hepatitis, Autoimmune 43 71
Aih 52 58
Autoimmune Hepatitis with Centrilobular Necrosis 12
Autoimmune Chronic Active Hepatitis 12
Autoimmune Chronic Hepatitis 52
Hepatitis Autoimmune 54

Characteristics:

Orphanet epidemiological data:

58
autoimmune hepatitis
Inheritance: Not applicable; Prevalence: 1-9/1000000 (Europe),1-9/1000000 (Norway),1-5/10000 (Norway),1-9/1000000 (Sweden),1-5/10000 (New Zealand),1-9/1000000 (Spain),1-5/10000 (Spain),1-5/10000 (Sweden),1-9/1000000 (Israel),1-5/10000 (Israel),1-9/100000 (Denmark),1-5/10000 (Denmark),1-9/100000 (Singapore),1-9/100000; Age of onset: Adult;

Classifications:

Orphanet: 58  
Rare hepatic diseases


Summaries for Autoimmune Hepatitis

KEGG : 36 Autoimmune hepatitis (AIH) is a chronic inflammatory liver disorder with an etiology that remains unclear. The pathogenesis may be a result of alterations in immune tolerance, a genetic predisposition and environmental conditions, which in collaboration induce a T-cell-mediated attack on liver antigens, leading to necro-inflammation and liver damage. AIH has marked female predilection. AIH can present at all ages, but the two peak ages of incidence are in childhood or adolescence and at around 40 years of age. Trademark biochemical/serological characteristics of AIH are elevated aminotransferase levels, positivity for autoantibodies and increased IgG. A histological picture of interface hepatitis is typical of AIH. The value of immunosuppressive therapy with glucocorticoids, alone or in combination with azathioprine, was well documented in numerous studies of AIH.

MalaCards based summary : Autoimmune Hepatitis, also known as hepatitis, autoimmune, is related to autoimmune hepatitis type 1 and sclerosing cholangitis. An important gene associated with Autoimmune Hepatitis is SEPSECS (Sep (O-Phosphoserine) TRNA:Sec (Selenocysteine) TRNA Synthase), and among its related pathways/superpathways are Allograft rejection and ICos-ICosL Pathway in T-Helper Cell. The drugs Tacrolimus and chloroquine have been mentioned in the context of this disorder. Affiliated tissues include Blood and Umbilical Cord, and related phenotypes are increased circulating igg level and elevated hepatic transaminase

Disease Ontology : 12 An autoimmune disease of gastrointestinal tract that results in inflammation located in liver caused by the body's immune system attacking the liver cells.

NIH Rare Diseases : 52 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 likely results from a combination of autoimmunity, environmental triggers, and a genetic predisposition . It occurs more frequently in females. Treatment typically includes corticosteroids and medications that suppress the immune system. In severe cases, a liver transplant may be needed.

Wikipedia : 74 Autoimmune hepatitis, formerly called lupoid hepatitis, is a chronic, autoimmune disease of the liver... more...

Related Diseases for Autoimmune Hepatitis

Diseases in the Hepatitis family:

Hepatitis C Virus Hepatitis a
Hepatitis C Viral Hepatitis
Hepatitis B Hepatitis D
Autoimmune Hepatitis Hepatitis E
Autoimmune Hepatitis Type 2 Autoimmune Hepatitis Type 1

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

(show top 50) (show all 647)
# Related Disease Score Top Affiliating Genes
1 autoimmune hepatitis type 1 34.6 HLA-DRB1 HLA-DQB1 GPT
2 sclerosing cholangitis 31.6 HLA-DRB1 GPT GGT1 F2 CD40LG
3 hepatitis 31.4 SEPSECS KRT8 IFNA1 GPT GOLM1 GGT1
4 hepatitis a 31.3 SEPSECS IFNA1 GPT GGT1 F2 CTLA4
5 liver cirrhosis 31.3 KRT8 IFNA1 HLA-DRB1 GPT GGT1 F2
6 cholangitis 31.2 HLA-DRB1 GPT GGT1 F2 DLAT CTLA4
7 hashimoto thyroiditis 31.1 HLA-DRB1 HLA-DQB1 CTLA4 AIRE
8 portal hypertension 31.1 GPT F2 CD40LG
9 hepatitis c 31.1 KRT8 IFNA1 GPT GGT1
10 lupus erythematosus 31.0 PDCD1 HLA-DRB1 HLA-DQB1 CTLA4 CD40LG
11 esophageal varix 31.0 GPT GGT1 F2
12 graves' disease 30.9 HLA-DRB1 HLA-DQB1 HLA-A CTLA4 AIRE
13 graft-versus-host disease 30.9 HLA-DRB1 HLA-DQB1 HLA-A
14 autoimmune pancreatitis 30.9 HLA-DRB1 CTLA4 CD40LG
15 microscopic polyangiitis 30.8 HLA-DRB1 CTLA4
16 drug-induced hepatitis 30.8 SEPSECS GPT F2 CYP2D6
17 bilirubin metabolic disorder 30.8 GPT GGT1 F2 CD40LG
18 ascending cholangitis 30.8 GPT F2
19 liver disease 30.7 SEPSECS KRT8 IFNA1 GPT GOLM1 GGT1
20 cholangitis, primary sclerosing 30.7 SEPSECS HLA-A GPT GGT1 F2 DLAT
21 vitiligo-associated multiple autoimmune disease susceptibility 1 30.7 PDCD1 CTLA4 AIRE
22 myasthenia gravis 30.7 HLA-DQB1 CTLA4 CD40LG AIRE
23 sarcoidosis 1 30.7 HLA-DRB1 HLA-DQB1 HLA-A CD40LG
24 biliary atresia 30.7 GPT GGT1 F2
25 viral hepatitis 30.6 SEPSECS KRT8 IFNA1 HLA-DRB1 GPT GGT1
26 hepatitis e 30.6 GPT GGT1 F2 CD40LG ASGR2
27 leishmaniasis 30.6 HLA-DRB1 HLA-DQB1 GPT CTLA4 ARG1
28 calcinosis 30.6 HLA-DRB1 HLA-DQB1 GPT
29 obstructive jaundice 30.5 GPT GGT1 F2
30 temporal arteritis 30.5 HLA-DRB1 CTLA4 CD40LG
31 acute cystitis 30.5 GPT F2 CD40LG
32 amelogenesis imperfecta, type ie 30.5 SEPSECS HLA-DRB1 FTCD CYP2D6
33 acute cholangitis 30.5 GPT GGT1 F2
34 juvenile rheumatoid arthritis 30.4 HLA-DRB1 HLA-DQB1 CTLA4
35 alpha-1-antitrypsin deficiency 30.4 SEPSECS GPT F2
36 celiac disease 1 30.4 IFNA1 HLA-DRB1 HLA-DQB1 HLA-A CTLA4 CD40LG
37 cryptogenic cirrhosis 30.4 KRT8 F2
38 hypothyroidism 30.4 IFNA1 GPT F2 CTLA4 AIRE
39 hepatitis b 30.4 IFNA1 GPT GGT1 F2 CTLA4 ASGR2
40 measles 30.4 IFNA1 HLA-DRB1 HLA-A CD40LG
41 neutropenia 30.3 TPMT IFNA1 GPT CD40LG
42 pericardial effusion 30.3 GPT F2 CD40LG
43 alcoholic hepatitis 30.3 GPT GGT1 F2
44 hepatitis d 30.3 IFNA1 GPT F2 CYP2D6 CD40LG
45 common variable immunodeficiency 30.3 HLA-DRB1 HLA-DQB1 HLA-A CTLA4 CD40LG
46 chickenpox 30.2 IFNA1 CTLA4 CD40LG
47 pemphigus 30.2 HLA-DRB1 HLA-DQB1 CD40LG
48 pharyngitis 30.2 HLA-DRB1 GPT CD40LG
49 autoimmune disease 30.2 TPMT PDCD1 IFNA1 HLA-DRB1 HLA-DQB1 HLA-A
50 panuveitis 30.2 HLA-DRB1 HLA-DQB1 CD40LG

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



Diseases related to Autoimmune Hepatitis

Symptoms & Phenotypes for Autoimmune Hepatitis

Human phenotypes related to Autoimmune Hepatitis:

58 31 (show all 32)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 increased circulating igg level 58 31 obligate (100%) Obligate (100%) HP:0003237
2 elevated hepatic transaminase 58 31 hallmark (90%) Very frequent (99-80%) HP:0002910
3 antinuclear antibody positivity 58 31 hallmark (90%) Very frequent (99-80%) HP:0003493
4 smooth muscle antibody positivity 58 31 hallmark (90%) Very frequent (99-80%) HP:0003262
5 antineutrophil antibody positivity 58 31 hallmark (90%) Very frequent (99-80%) HP:0003453
6 liver kidney microsome type 1 antibody positivity 58 31 hallmark (90%) Very frequent (99-80%) HP:0030908
7 anti-liver cytosolic antigen type 1 antibody positivity 58 31 hallmark (90%) Very frequent (99-80%) HP:0030909
8 arthralgia 58 31 frequent (33%) Frequent (79-30%) HP:0002829
9 abdominal pain 58 31 frequent (33%) Frequent (79-30%) HP:0002027
10 depressivity 58 31 frequent (33%) Frequent (79-30%) HP:0000716
11 chronic fatigue 58 31 frequent (33%) Frequent (79-30%) HP:0012432
12 spider hemangioma 58 31 frequent (33%) Frequent (79-30%) HP:0012522
13 splenomegaly 58 31 occasional (7.5%) Occasional (29-5%) HP:0001744
14 arthritis 58 31 occasional (7.5%) Occasional (29-5%) HP:0001369
15 ascites 58 31 occasional (7.5%) Occasional (29-5%) HP:0001541
16 cirrhosis 58 31 occasional (7.5%) Occasional (29-5%) HP:0001394
17 gastrointestinal hemorrhage 58 31 occasional (7.5%) Occasional (29-5%) HP:0002239
18 anxiety 58 31 occasional (7.5%) Occasional (29-5%) HP:0000739
19 vitiligo 58 31 occasional (7.5%) Occasional (29-5%) HP:0001045
20 thyroiditis 58 31 occasional (7.5%) Occasional (29-5%) HP:0100646
21 jaundice 58 31 occasional (7.5%) Occasional (29-5%) HP:0000952
22 increased total bilirubin 58 31 occasional (7.5%) Occasional (29-5%) HP:0003573
23 glomerulonephritis 58 31 occasional (7.5%) Occasional (29-5%) HP:0000099
24 acute hepatitis 58 31 occasional (7.5%) Occasional (29-5%) HP:0200119
25 ulcerative colitis 58 31 occasional (7.5%) Occasional (29-5%) HP:0100279
26 diffuse hepatic steatosis 58 31 occasional (7.5%) Occasional (29-5%) HP:0006555
27 sclerosing cholangitis 58 31 occasional (7.5%) Occasional (29-5%) HP:0030991
28 hepatocellular carcinoma 58 31 very rare (1%) Very rare (<4-1%) HP:0001402
29 viral hepatitis 58 31 very rare (1%) Very rare (<4-1%) HP:0006562
30 fulminant hepatitis 58 31 very rare (1%) Very rare (<4-1%) HP:0004787
31 inflammation of the large intestine 58 Occasional (29-5%)
32 increased antibody level in blood 58 Obligate (100%)

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

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased viability GR00240-S-1 9.66 CD40LG
2 Decreased viability GR00249-S 9.66 DLAT GGT1 HLA-A PDCD1 TPMT
3 Decreased viability GR00381-A-1 9.66 CYP2D6 DLAT GGT1 HLA-DQB1 PDCD1
4 Decreased viability GR00381-A-3 9.66 HLA-DQB1 PDCD1
5 Decreased viability GR00386-A-1 9.66 ASGR2 GOLM1 HLA-A HLA-DRB1
6 Decreased viability GR00402-S-2 9.66 CYP2D6 GPT HLA-DQB1 KRT8 PDCD1

MGI Mouse Phenotypes related to Autoimmune Hepatitis:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 10.03 AIRE ARG1 ASGR2 CD40LG CTLA4 F2
2 immune system MP:0005387 9.93 AIRE ARG1 ASGR2 CD40LG CTLA4 F2
3 liver/biliary system MP:0005370 9.5 AIRE ARG1 CTLA4 GOLM1 HLA-DQB1 KRT8
4 mortality/aging MP:0010768 9.47 AIRE ARG1 ASGR2 CD40LG CTLA4 DLAT

Drugs & Therapeutics for Autoimmune Hepatitis

Drugs for Autoimmune Hepatitis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 113)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Tacrolimus Approved, Investigational Phase 4 104987-11-3 445643 439492 6473866
2
chloroquine Approved, Investigational, Vet_approved Phase 4 54-05-7 2719
3
Sorafenib Approved, Investigational Phase 4 284461-73-0 216239 406563
4
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 6741
5
Methylprednisolone hemisuccinate Approved Phase 4 2921-57-5
6
Prednisolone phosphate Approved, Vet_approved Phase 4 302-25-0
7
Mycophenolic acid Approved Phase 4 24280-93-1 446541
8
Prednisolone Approved, Vet_approved Phase 4 50-24-8 5755
9 Prednisolone acetate Approved, Vet_approved Phase 4 52-21-1
10
Clotrimazole Approved, Vet_approved Phase 4 23593-75-1 2812
11
Miconazole Approved, Investigational, Vet_approved Phase 4 22916-47-8 4189
12
Vitamin D3 Approved, Nutraceutical Phase 4 67-97-0 5280795 6221
13
Ornithine Approved, Nutraceutical Phase 4 70-26-8, 3184-13-2 6262
14
Aspartic acid Approved, Nutraceutical Phase 4 56-84-8 5960
15
Prednisolone hemisuccinate Experimental Phase 4 2920-86-7
16 Analgesics Phase 4
17 Analgesics, Non-Narcotic Phase 4
18 Anti-Inflammatory Agents Phase 4
19 Antirheumatic Agents Phase 4
20 Anti-Inflammatory Agents, Non-Steroidal Phase 4
21 Calcineurin Inhibitors Phase 4
22 Pharmaceutical Solutions Phase 4
23 Hormones Phase 4
24 Antineoplastic Agents, Hormonal Phase 4
25 glucocorticoids Phase 4
26 Hormone Antagonists Phase 4
27 Amebicides Phase 4
28 Anthelmintics Phase 4
29 Chloroquine diphosphate Phase 4 50-63-5
30 Antimalarials Phase 4
31 Trace Elements Phase 4
32 Micronutrients Phase 4
33 Dihydroxycholecalciferols Phase 4
34 Nutrients Phase 4
35 Calcium, Dietary Phase 4
36 Neurotransmitter Agents Phase 4
37 N-Methylaspartate Phase 4
38 Antioxidants Phase 4
39 Protective Agents Phase 4
40 silymarin Phase 4
41 Liver Extracts Phase 4
42 Protein Kinase Inhibitors Phase 4
43 Methylprednisolone Acetate Phase 4
44 Antitubercular Agents Phase 4
45 Anti-Infective Agents Phase 4
46 Cyclosporins Phase 4
47 Dermatologic Agents Phase 4
48 Antifungal Agents Phase 4
49 Anti-Bacterial Agents Phase 4
50 Antibiotics, Antitubercular Phase 4

Interventional clinical trials:

(show top 50) (show all 75)
# Name Status NCT ID Phase Drugs
1 A Randomised, Open-label Clinical Trial Assessing the Efficacy and Safety of Mycophenolate Mofetil Versus Azathioprine for Induction of Remission in Treatment Naive Autoimmune Hepatitis Unknown status NCT02900443 Phase 4 Mycophenolate mofetil;Azathioprine
2 Mycophenolate vs Azathioprin in Autoimmune Hepatitis Completed NCT00687180 Phase 4 Treatment with mycophenolat mofetil;Treatment with azathioprin
3 DOUBLE-BLIND RANDOMIZED CLINICAL TRIAL WITH CHLOROQUINE VERSUS PLACEBO FOR MAINTENANCE OF REMISSION OF AUTOIMMUNE HEPATITIS Completed NCT01980745 Phase 4 Chloroquine diphosphate 250mg;Placebo
4 Possible Role of Chloroquine in Conjunction to Prednisone to Induce a Complete Remission in the Treatment of Autoimmune Hepatitis: a Randomized Trial Completed NCT02463331 Phase 4 Chloroquine diphosphate;prednisone;azathioprine
5 Use of Vitamin D in Treatment of Non-Alcoholic Fatty Liver Disease Detected by Transient Elastography Completed NCT04038853 Phase 4 1,25-Dihydroxyvitamin D;Placebo
6 Influence of Methylprednisolone Pulse Therapy on Liver Function in Patients With Graves' Orbitopathy Completed NCT03667157 Phase 4 every week IVMP therapy;very high doses IVMP therapy
7 Comparison Of Efficacy Of Two Immunosuppressive Protocols Including Tacrolimus With Or Without Mycophenolate Mofetil In Pediatric Liver Transplantation Aimed In Early Termination Of Steroid Therapy Completed NCT00195988 Phase 4 tacrolimus, steroids, mycophenolate mofetil
8 Efficacy of Intravenous 'L-ornithine L-aspartate' in Reversal of Overt Acute Hepatic Encephalopathy in Patients With Liver Cirrhosis: a Prospective, Randomized, Double-blind, Placebo Controlled Trial Completed NCT01722578 Phase 4 L-ornithine L-aspartate;Placebo
9 Silymarin® - Efficacy in Treatment of Non-alcoholic Fatty Liver Disease (NAFLD) Controlled by Laboratory and and Elastographic Parameters Recruiting NCT02973295 Phase 4 Silymarin
10 Mechanism of Sorafenib Resistance in Patients With Advanced Hepatocellular Carcinoma Recruiting NCT02733809 Phase 4 Sorafenib
11 Mycophenolate Mofetil Versus Cyclosporin A in the Treatment of Primary Biliary Cholangitis-autoimmune Hepatitis Overlap Syndrome Duo to Nonresponse to Standard Therapy Not yet recruiting NCT04376528 Phase 4 Cyclosporin A;Mycophenolate Mofetil
12 Comparing Efficacy and Tolerability of Cyclosporine-A vs. Prednisolone for Induction of Remission in Auto-immune Hepatitis Completed NCT01170351 Phase 3 Cyclosporine-A
13 Efficacy and Safety of Budesonide Capsules (3x3mg/d)Versus Prednisone in Patients With a Diagnose of Active Autoimmune Hepatitis. A Double-blind, Randomized, Active-controlled, Multicentre Study Completed NCT00838214 Phase 2, Phase 3 budesonide;prednisone
14 A Phase 3 Study to Evaluate the Place of Budesonide in the Treatment of GVHD Completed NCT00128739 Phase 3 Budesonide
15 A Randomized, Double-blind, Placebo-controlled Study of the Safety and Efficacy of VAY736 in Autoimmune Hepatitis Recruiting NCT03217422 Phase 2, Phase 3
16 Comparison of Bovine Colostrum Versus Placebo in Treatment of Severe Alcoholic Hepatitis: A Randomized Double Blind Controlled Trial Recruiting NCT02473341 Phase 3 Bovine Colostrum;Placebo
17 A Seamless Phase II/III 2-stage Design, Randomized, Double-Blind, Placebo Controlled, Trial of JKB-122 as an Adjunct Therapy to Prednisolone and Azathioprine in the Induction of Remission in Autoimmune Hepatitis (AIH) Not yet recruiting NCT04371718 Phase 2, Phase 3 JKB-122
18 Therapeutic Plasma Exchange in Patients With Acute on Chronic Liver Failure: A Randomized Controlled Trial (PLEXAR) Not yet recruiting NCT04051437 Phase 3 Standard medical treatment
19 Double Blind, Multicentric, Randomized, Placebo-controlled Trial, Evaluating the Efficacy of 24 Month of Bezafibrate in Primary Sclerosing Cholangitis With Persistent Cholestasis Despite Ursodeoxycholic Acid Therapy Not yet recruiting NCT04309773 Phase 3 Bezafibrate (400mg) in addition to standard 15-20 mg/kg/jour UDCA therapy;Placebo of Bezafibrate in addition to standard UDCA therapy
20 Paeoniflorin Combination of Hepatoprotective Drugs Versus Hepatoprotective Drugs Only for Treatment of Auto-immune Hepatitis Withdrawn NCT02878863 Phase 3 Paeoniflorin + phosphatidylcholine or silymarin;Phosphatidylcholine or silymarin
21 Phase 1 Clinical Trial Using Regulatory T Cells as Individualized Medicine to Evaluate the Safety and Efficacy in Autoimmune Hepatitis Unknown status NCT02704338 Phase 1, Phase 2
22 Phase 1/2 Study of UC-MSC Treatment for Evaluation the Efficacy and Safety in Patients With Autoimmune Liver Disease Unknown status NCT01661842 Phase 1, Phase 2
23 A Phase 2, Pilot Study of JKB-122 to Assess Liver Tests (ALT) in Autoimmune Hepatitis (AIH) Patients Who Are Refractory or Intolerant to Current Therapies Completed NCT02556372 Phase 2 JKB-122
24 Efficacy of Combination Therapy of Glucocorticoids, and Bovine Colostrum in Treatment of Severe Alcoholic Hepatitis: A Pilot Study. Completed NCT02265328 Phase 2
25 A Phase 2a Study to Evaluate the Safety and Tolerability of OCR-002 (Ornithine Phenylacetate) in the Treatment of Patients With Acute Liver Failure/Severe Acute Liver Injury Completed NCT01548690 Phase 2 Ornithine Phenylacetate
26 A Pilot Study to Evaluate the Efficacy and Safety of Budesonide as an Alternative to Prednisone for Liver Transplant Immune Suppression Completed NCT03304626 Phase 2 Budesonide 3Mg Capsule
27 Induction of Regulatory t Cells by Low Dose IL2 in Autoimmune and Inflammatory Diseases: a Transnosographic Approach Recruiting NCT01988506 Phase 2 Interleukin 2
28 An Adaptive,Multicentre, Phase IIa, Multi-disease Trial Investigating the Safety & Activity of a Single Infusion of Selected Mesenchymal Stromal Cells in the Treatment of Patients With Primary Sclerosing Cholangitis & Autoimmune Hepatitis Recruiting NCT02997878 Phase 1, Phase 2
29 A Single-center, Open Label, Cross-over Study on the Effects of Ursodeoxycholic Acid (UDCA) in Patients With Hepatic Sarcoidosis Active, not recruiting NCT03602976 Phase 2 Ursodeoxycholic Acid
30 A Phase II, Open-Label, Multi-Center, Prospective, Randomized Study of LCP-Tacro Tablets vs. Azathioprine, in Combination With Corticosteroids, for the Treatment of Autoimmune Hepatitis Terminated NCT00608894 Phase 2 LCP-Tacro (tacrolimus);Azathioprine
31 A Phase 2a Open Label, Single Arm Study to Evaluate the Safety and Efficacy of Cannabidiol Only as Maintenance Therapy and Steroid Sparing in Patients With Stable Autoimmune Hepatitis Terminated NCT04129489 Phase 2 Cannabidiol
32 Phase II, Open Label, Adaptive Design, Multiple Dose Finding Study to Investigate Synthetic PreImplantation Factor (sPIF) in Patients With Autoimmune Hepatitis (AIH) Withdrawn NCT03593460 Phase 2 Synthetic PreImplantation Factor
33 A Phase 1, Randomized, Double-Blind, Placebo-Controlled, Single and Multiple Ascending Dose Study to Investigate the Safety, Tolerability and Pharmacokinetics of Synthetic PreImplantation Factor (sPIF) in Autoimmune Hepatitis Completed NCT02239562 Phase 1 sPIF;Placebo
34 Ursodeoxycholic Acid Therapy in Pediatric Primary Sclerosing Cholangitis: A Pilot Withdrawal/Reinstitution Trial Completed NCT01088607 Phase 1 ursodeoxycholic acid (UDCA)
35 Abatacept for Treatment of Recurrent or de Novo Autoimmune Hepatitis Recruiting NCT04203875 Phase 1 Orencia® (Abatacept)
36 A Randomized, Open-label, Multiple-dose, Two-period, Crossover, Study to Investigate the Pharmacokinetic Profile of 2 Formulations of JKB-122 in Healthy Male Subjects Not yet recruiting NCT04313205 Phase 1 JKB-122 in capsule or tablet form
37 Differential Gene Expression in Liver Tissue and Blood From Individuals With Chronic Viral Hepatitis With or Without a Complicating Hepatoma or Autoimmune Liver Disease Unknown status NCT00160940
38 Serial HBV DNA Levels During Pregnancy in Patients With Chronic Hepatitis B: a Prospective Observational Follow-up Study Unknown status NCT01610115
39 A Study Of Metabolic Factors And Efficacy Of Pentoxifylline Versus Pioglitazone In Lean And Obese Nash (Non-Alcoholic Steatohepatitis) Patients. Unknown status NCT00681733 Pioglitazone;Pioglitazone;Pentoxifylline
40 Multicentric Study on the Long-term Follow-up of Autoimmune Hepatitis in the Pediatric Patients Completed NCT04247490
41 Prospective Comparison of Transient Elastography and Mini-laparoscopic Guided Liver Biopsy in Autoimmune Hepatitis Completed NCT01905254
42 Effect of a High-protein High-fiber Diet and Nutritional Status, Serum Ammonia Concentration and Plasma Cytokines in Patients With Autoimmune Hepatitis Completed NCT01655121
43 Assessing Kids for Liver Inflammation and Fibrosis Using Non-invasive MRI Completed NCT03198104
44 Quantitative Magnetic Resonance Imaging to Aid Clinical Decision Making in Autoimmune Hepatitis. Completed NCT03979053
45 A Retrospective, Open-label, Uncontrolled Cohort Study to Assess the Effectiveness and Safety of Lactulose Retention Enema in the Treatment of Cirrhotic Patients With Grade 3 or 4 (West Haven Criteria) Hepatic Encephalopathy (HE) [The RETRO Study] Completed NCT02377947
46 Pruritus and Melanoma and Cutaneous Carcinomas Treated With Immunotherapy (PRURICHECKPOINT) Completed NCT04365244
47 A Correlative Study of Melanoma Tumor-Infiltrating Lymphocytes (TILs) and Response to Ipilimumab Completed NCT01715077 Ipilimumab at 3 mg/kg dose
48 The Curative Effect and Security of Entecavir Combined Thymosin or Resveratrol on HBeAg Positive Chronic Hepatitis B Patients - a Multi-center, Random, Control, Open Clinical Trial Completed NCT03509688 entecavir+resveratrol;Entecavir;entecavir+thymosin α1
49 The Curative Effect and Security of Interferon Combined Resveratrol on HBeAg Positive Chronic Hepatitis B Patients - a Multi-center, Random, Control, Open Clinical Trial. Completed NCT03546530 Interferon;Interferon+resveratrol
50 Plasma Exchange Combination of Immunosuppressive Regimens for the Remission of Auto-immune Hepatitis Recruiting NCT02874586

Search NIH Clinical Center for Autoimmune Hepatitis

Cell-based therapeutics:


LifeMap Discovery
Data from LifeMap, the Embryonic Development and Stem Cells Database
Read about Autoimmune Hepatitis cell therapies at LifeMap Discovery.

Cochrane evidence based reviews: hepatitis, autoimmune

Genetic Tests for Autoimmune Hepatitis

Genetic tests related to Autoimmune Hepatitis:

# Genetic test Affiliating Genes
1 Autoimmune Hepatitis 29

Anatomical Context for Autoimmune Hepatitis

The Foundational Model of Anatomy Ontology organs/tissues related to Autoimmune Hepatitis:

19
Liver

MalaCards organs/tissues related to Autoimmune Hepatitis:

40
Liver, T Cells, Testes, Kidney, Thyroid, Bone, Smooth Muscle
LifeMap Discovery
Data from LifeMap, the Embryonic Development and Stem Cells Database

Cells/anatomical compartments in embryo or adult related to Autoimmune Hepatitis:
# Tissue Anatomical CompartmentCell Relevance
1 Blood Hematopoietic Bone Marrow Hematopoietic Stem Cells Potential therapeutic candidate
2 Umbilical Cord Wharton's Jelly Mesenchymal Stem Cells Potential therapeutic candidate

Publications for Autoimmune Hepatitis

Articles related to Autoimmune Hepatitis:

(show top 50) (show all 5573)
# Title Authors PMID Year
1
Viral triggers for autoimmunity: is the 'glass of molecular mimicry' half full or half empty? 54 61
19716269 2010
2
Characterisation and utility of thiopurine methyltransferase and thiopurine metabolite measurements in autoimmune hepatitis. 61 54
19906459 2010
3
LKM-1 sera from autoimmune hepatitis patients that recognize ERp57, carboxylesterase 1 and CYP2D6. 61 54
20208391 2010
4
Diagnostic utility of IgG and IgM immunohistochemistry in autoimmune liver disease. 54 61
20101770 2010
5
Cytochrome P450 2D6 as a model antigen. 61 54
20460894 2010
6
Autoantibodies to asialoglycoprotein receptor (ASGPR) measured by a novel ELISA--revival of a disease-activity marker in autoimmune hepatitis. 61 54
19576873 2009
7
Study of nonstandard auto-antibodies as prognostic markers in auto immune hepatitis in children. 61 54
19712439 2009
8
Immune responses in autoimmune hepatitis: effect of prednisone and azathioprine treatment: case report. 54 61
19584951 2009
9
AIRE gene analysis in children with autoimmune hepatitis type I or II. 54 61
19322061 2009
10
[Optimising azathioprine treatment: determination of thiopurine methyltransferase activity and thiopurine metabolites]. 61 54
19217567 2009
11
HLA and autoimmune digestive disease: a clinically oriented review for gastroenterologists. 61 54
19098870 2009
12
Decreased production of immunoglobulin M and A in autoimmune pancreatitis. 61 54
19626266 2009
13
Interferon (IFN) for malignant melanoma unmasking an autoimmune hepatitis. 61 54
19237078 2009
14
Identification of CD4 T-cell epitopes in soluble liver antigen/liver pancreas autoantigen in autoimmune hepatitis. 54 61
18773898 2008
15
Effects of serum aspartate aminotransferase levels in patients with autoimmune hepatitis influence disease course and outcome. 61 54
18840547 2008
16
Graft histology characteristics in long-term survivors of pediatric liver transplantation. 61 54
18975292 2008
17
Novel homozygous AIRE mutation in a German patient with severe APECED. 61 54
19209622 2008
18
A comparative study of endoscopic ultrasonography versus endoscopic retrograde cholangiopancreatography in children with chronic liver disease. 54 61
18923212 2008
19
Deficiency of forkhead box P3 and cytotoxic T-lymphocyte-associated antigen-4 gene expressions and impaired suppressor function of CD4(+)CD25(+) T cells in patients with autoimmune hepatitis. 61 54
18624718 2008
20
An 8-year-old boy with autoimmune hepatitis and Candida onychosis as the first symptoms of autoimmune polyglandular syndrome (APS1): identification of a new homozygous mutation in the autoimmune regulator gene (AIRE). 54 61
17891543 2008
21
Association of cytotoxic T-lymphocyte antigen 4 gene polymorphisms with type 1 autoimmune hepatitis in Japanese. 61 54
18371160 2008
22
Ballooned hepatocytes in steatohepatitis: the value of keratin immunohistochemistry for diagnosis. 54 61
18329127 2008
23
Enzymes as target antigens of liver-specific autoimmunity: the case of cytochromes P450s. 61 54
18781950 2008
24
Association of CTLA4 single nucleotide polymorphisms with viral but not autoimmune liver disease. 61 54
18049163 2007
25
Cytochrome P450IID6-specific CD8 T cell immune responses mirror disease activity in autoimmune hepatitis type 2. 61 54
17559153 2007
26
Intrahepatic expression of the co-stimulatory molecules programmed death-1, and its ligands in autoimmune liver disease. 54 61
17610472 2007
27
Fulminant hepatic failure in a case of autoimmune hepatitis in hepatitis C during peg-interferon-alpha 2b plus ribavirin treatment. 61 54
17708618 2007
28
Selenocysteine, soluble liver antigen/liver-pancreas, and autoimmune hepatitis. 61 54
17596869 2007
29
Combination of tumor site-located CTL-associated antigen-4 blockade and systemic regulatory T-cell depletion induces tumor-destructive immune responses. 54 61
17575163 2007
30
Post-translational methylation of high mobility group box 1 (HMGB1) causes its cytoplasmic localization in neutrophils. 61 54
17403684 2007
31
Recurrence of autoimmune hepatitis after liver transplantation without elevation of alanine aminotransferase. 54 61
17461459 2007
32
Mapping of human autoantibody epitopes on aromatic L-amino acid decarboxylase. 61 54
17200166 2007
33
A functional Fas promoter polymorphism is associated with a severe phenotype in type 1 autoimmune hepatitis characterized by early development of cirrhosis. 61 54
17493146 2007
34
Pathogenesis of autoimmune hepatitis: from break of tolerance to immune-mediated hepatocyte apoptosis. 61 54
17320796 2007
35
Thiopurine S-methyltransferase gene polymorphism in Japanese patients with autoimmune liver diseases. 61 54
17241387 2007
36
Cytokeratin 8 and 18 expression in imprint smears of chronic viral hepatitis, autoimmune hepatitis and hepatocellular carcinoma. A preliminary study. 54 61
17328497 2007
37
PD-L1 is induced in hepatocytes by viral infection and by interferon-alpha and -gamma and mediates T cell apoptosis. 54 61
16876901 2006
38
Utility of thiopurine methyltransferase genotyping and phenotyping, and measurement of azathioprine metabolites in the management of patients with autoimmune hepatitis. 61 54
16876902 2006
39
Multiplex neuritis in a patient with autoimmune hepatitis: a case report. 61 54
16981276 2006
40
Serum reactivity against bacterial pyruvate dehydrogenase: increasing the specificity of anti-mitochondrial antibodies for the diagnosis of primary biliary cirrhosis. 61 54
17162370 2006
41
Thiopurine methyltransferase deficiency and azathioprine intolerance in autoimmune hepatitis. 54 61
16773433 2006
42
Polyclonal T-cell responses to cytochrome P450IID6 are associated with disease activity in autoimmune hepatitis type 2. 54 61
16530525 2006
43
[A retrospective study on the role of antibodies against soluble liver antigen (anti-SLA antibodies) and other autoantibodies in the diagnostics of autoimmune hepatitis]. 54 61
16553048 2006
44
Autoimmune hepatitis, from mechanisms to therapy. 54 61
16447290 2006
45
Cost-effectiveness of pharmacotherapy for autoimmune hepatitis. 54 61
16433580 2006
46
Rapid-onset primary biliary cirrhosis resembling drug-induced liver injury. 61 54
16293915 2005
47
LKM1 autoantibodies in chronic hepatitis C infection: a case of molecular mimicry? 61 54
16037945 2005
48
C77G mutation in protein tyrosine phosphatase CD45 gene and autoimmune hepatitis. 61 54
15963758 2005
49
Susceptibility to thyroid disorders in hepatitis C. 54 61
15952102 2005
50
Fas polymorphisms influence susceptibility to autoimmune hepatitis. 54 61
15929764 2005

Variations for Autoimmune Hepatitis

Expression for Autoimmune Hepatitis

Search GEO for disease gene expression data for Autoimmune Hepatitis.

Pathways for Autoimmune Hepatitis

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

# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.17 IFNA1 HLA-DRB1 HLA-DQB1 HLA-A CTLA4 CD40LG
2
Show member pathways
12 HLA-DRB1 HLA-DQB1 CTLA4 CD40LG
3
Show member pathways
11.69 PDCD1 HLA-DRB1 HLA-DQB1 CTLA4
4 11.51 HLA-DRB1 HLA-DQB1 CTLA4
5 11.28 HLA-DRB1 HLA-DQB1 CTLA4
6 11.27 PDCD1 HLA-DRB1 HLA-DQB1 HLA-A CTLA4 CD40LG

GO Terms for Autoimmune Hepatitis

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

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.86 IFNA1 HLA-DRB1 GPT GOLM1 GGT1 F2
2 external side of plasma membrane GO:0009897 9.35 PDCD1 HLA-DRB1 F2 CTLA4 CD40LG
3 ER to Golgi transport vesicle membrane GO:0012507 9.33 HLA-DRB1 HLA-DQB1 HLA-A
4 integral component of lumenal side of endoplasmic reticulum membrane GO:0071556 8.8 HLA-DRB1 HLA-DQB1 HLA-A

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

(show all 12)
# Name GO ID Score Top Affiliating Genes
1 T cell receptor signaling pathway GO:0050852 9.67 HLA-DRB1 HLA-DQB1 HLA-A CTLA4
2 interferon-gamma-mediated signaling pathway GO:0060333 9.65 HLA-DRB1 HLA-DQB1 HLA-A
3 humoral immune response GO:0006959 9.63 PDCD1 IFNA1 AIRE
4 T cell costimulation GO:0031295 9.58 PDCD1 CTLA4 CD40LG
5 negative regulation of immune response GO:0050777 9.51 PDCD1 CTLA4
6 negative regulation of T cell proliferation GO:0042130 9.5 HLA-DRB1 CTLA4 ARG1
7 detection of bacterium GO:0016045 9.49 HLA-DRB1 HLA-A
8 humoral immune response mediated by circulating immunoglobulin GO:0002455 9.43 HLA-DRB1 HLA-DQB1
9 antigen processing and presentation GO:0019882 9.43 HLA-DRB1 HLA-DQB1 HLA-A
10 immune response GO:0006955 9.43 HLA-DRB1 HLA-DQB1 HLA-A CTLA4 CD40LG AIRE
11 immunoglobulin production involved in immunoglobulin mediated immune response GO:0002381 9.16 HLA-DRB1 HLA-DQB1
12 adaptive immune response GO:0002250 9.1 PDCD1 IFNA1 HLA-DRB1 HLA-A CTLA4 ARG1

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

# Name GO ID Score Top Affiliating Genes
1 MHC class II receptor activity GO:0032395 8.96 HLA-DRB1 HLA-DQB1
2 peptide antigen binding GO:0042605 8.8 HLA-DRB1 HLA-DQB1 HLA-A

Sources for Autoimmune Hepatitis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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