Autoimmune hepatitis

disease

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Also known as AIHautoimmune chronic active hepatitisautoimmune chronic hepatitisautoimmune hepatitis with centrilobular necrosisautoimmune liver diseasechronic autoimmune hepatitis

Summary

Autoimmune hepatitis (MONDO:0016264) is a disease (an umbrella term covering 7 Mondo subtypes) with 4 cohort genes (17 GWAS associations across 6 studies) and 64 clinical trials. Top therapeutic interventions include azathioprine, mycophenolate mofetil, and cannabidiol.

At a glance

Prevalence: 1-5 / 10 000 (Norway) [Orphanet-validated]
Umbrella term: 7 Mondo subtypes
Cohort genes: 4
GWAS associations: 17
Phenotypes (HPO): 34
Clinical trials: 64

Clinical features

Epidemiology

Prevalence records

17 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Annual incidence	1-9 / 1 000 000	0.75	Europe	Validated
Annual incidence	1-9 / 100 000	1.9	Norway	Validated
Annual incidence	1-9 / 1 000 000	0.85	Sweden	Validated
Annual incidence	1-9 / 1 000 000	0.95	Spain	Validated
Annual incidence	1-9 / 1 000 000	0.67	Israel	Validated
Annual incidence	1-9 / 100 000	1.68	Denmark	Validated
Point prevalence	1-5 / 10 000	16.9	Norway	Validated
Point prevalence	1-5 / 10 000	24.5	New Zealand	Validated
Point prevalence	1-5 / 10 000	11.6	Spain	Validated
Point prevalence	1-5 / 10 000	10.7	Sweden	Validated
Point prevalence	1-5 / 10 000	11	Israel	Validated
Point prevalence	1-5 / 10 000	23.9	Denmark	Validated
Point prevalence	1-9 / 100 000	4	Singapore	Validated
Point prevalence	1-5 / 10 000	42.9	Specific population	Validated
Annual incidence	1-9 / 100 000	1.2	Worldwide	Not yet validated
Point prevalence	1-5 / 10 000	23.5	Worldwide	Not yet validated
Point prevalence	1-5 / 10 000		Europe	Not yet validated

Signs & symptoms

Clinical features (HPO)

34 HPO clinical features (Orphanet curated; top 34 by frequency):

HPO ID	Term	Frequency
HP:0003237	Increased circulating IgG level	Obligate (100%)
HP:0010702	Increased circulating antibody level	Obligate (100%)
HP:0002910	Elevated circulating hepatic transaminase concentration	Very frequent (80-99%)
HP:0003262	Smooth muscle antibody positivity	Very frequent (80-99%)
HP:0003453	Antineutrophil antibody positivity	Very frequent (80-99%)
HP:0003493	Antinuclear antibody positivity	Very frequent (80-99%)
HP:0030908	Liver kidney microsome type 1 antibody positivity	Very frequent (80-99%)
HP:0030909	Anti-liver cytosolic antigen type 1 antibody positivity	Very frequent (80-99%)
HP:0031925	Rosette	Very frequent (80-99%)
HP:0000716	Depression	Frequent (30-79%)
HP:0002027	Abdominal pain	Frequent (30-79%)
HP:0002829	Arthralgia	Frequent (30-79%)
HP:0012432	Chronic fatigue	Frequent (30-79%)
HP:0012522	Spider hemangioma	Frequent (30-79%)
HP:0032469	Anti-asialoglycoprotein receptor antibody positivity	Frequent (30-79%)
HP:0000099	Glomerulonephritis	Occasional (5-29%)
HP:0000739	Anxiety	Occasional (5-29%)
HP:0000952	Jaundice	Occasional (5-29%)
HP:0001045	Vitiligo	Occasional (5-29%)
HP:0001369	Arthritis	Occasional (5-29%)
HP:0001394	Cirrhosis	Occasional (5-29%)
HP:0001541	Ascites	Occasional (5-29%)
HP:0001744	Splenomegaly	Occasional (5-29%)
HP:0002037	Inflammation of the large intestine	Occasional (5-29%)
HP:0002239	Gastrointestinal hemorrhage	Occasional (5-29%)
HP:0003573	Increased total bilirubin	Occasional (5-29%)
HP:0006555	Diffuse hepatic steatosis	Occasional (5-29%)
HP:0030991	Sclerosing cholangitis	Occasional (5-29%)
HP:0100279	Ulcerative colitis	Occasional (5-29%)
HP:0100646	Thyroiditis	Occasional (5-29%)
HP:0200119	Acute hepatitis	Occasional (5-29%)
HP:0001402	Hepatocellular carcinoma	Very rare (<1-4%)
HP:0004787	Fulminant hepatitis	Very rare (<1-4%)
HP:0006562	Viral hepatitis	Very rare (<1-4%)

Identifiers

Disease identifiers

Field	Value
Canonical name	autoimmune hepatitis
Mondo ID	MONDO:0016264
EFO	EFO:0005676
MeSH	D019693
Orphanet	2137
DOID	DOID:2048
ICD-10-CM	K75.4
ICD-11	1235727122
NCIT	C27029
SNOMED CT	408335007
UMLS	C4721555
MedGen	1666753
GARD	0005871
MedDRA	10003827
NORD	1897
Is cancer (heuristic)	no

Also known as: AIH · autoimmune chronic active hepatitis · autoimmune chronic hepatitis · autoimmune hepatitis · autoimmune hepatitis with centrilobular necrosis · autoimmune liver disease · chronic autoimmune hepatitis

Data availability: 17 GWAS associations (6 studies) · 24 cell lines.

Disease family

An umbrella term covering 7 Mondo subtypes.

Classification path: disease › human disease › disease by body system or component › endocrine system disorder › autoimmune disorder of endocrine system › autoimmune hepatitis

Subtypes (7): autoimmune hepatitis type 1, autoimmune hepatitis type 2, autoimmune hepatitis type 3, primary biliary cholangitis/primary sclerosing cholangitis and autoimmune hepatitis overlap syndrome, seronegative autoimmune hepatitis, autoimmune cholangitis, giant cell hepatitis with autoimmune hemolytic anemia

Genetics & variants

GWAS landscape

17 GWAS associations across 6 studies. Top hits map to 12 distinct genes (as reported by GWAS).

Top associations by p-value

rsID	p-value	Gene	Risk allele	Odds ratio
rs2187668	2e-78	HLA-DQA1	?	2.9
rs6932730	9e-73	MICA-AS1	?	2.32
rs1794514	6e-14	HLA-DQB1 - MTCO3P1	C	0.5
DQA1*05:01	4e-11		?
rs3891175	5e-11	HLA-DQB1	?	2.41
rs72929257	3e-09	ICOS - DSTNP5	?	1.31
rs6809477	5e-09	SYNPR-AS1, SYNPR	?	1.25
rs56036302	7e-09	TSBP1-AS1, TSBP1	T	1.24
rs564176274	9e-09	HLA-DQB1 - MTCO3P1	?	0.02
rs3184504	8e-08	ATXN2, SH2B3	?	1.4
rs11889341	1e-07	STAT4	?	1.24
DQB1*02:01	2e-07		?
rs9564997	3e-07	LINC00393	?	0.81
rs7574865	1e-06	STAT4	?	1.21
rs6000782	3e-06	CARD10 - CDC42EP1	?	1.7
rs11084330	5e-06	LILRA4	?	0.66
rs11117432	6e-06	LINC02132 - LINC01082	?	0.72

Top studies (by case count)

Study	Lead author	Year	Cases	Controls	Title
GCST90103934	Li Y	2022	1,622	10,466	Genome-wide Meta-analysis Identifies Novel Susceptibility Loci for Autoimmune Hepatitis Type 1.
GCST90018785	Sakaue S	2021	821	484,413	A cross-population atlas of genetic associations for 220 human phenotypes.
GCST002423	de Boer YS	2014	649	13,436	Genome-wide association study identifies variants associated with autoimmune hepatitis type 1.
GCST90492681	Lyu X	2024	153	212,616	A genome-wide cross-trait analysis identifying shared genetic basis and causal relationships between Hunner-type interstitial cystitis and autoimmune diseases in East Asian populations.
GCST005853	Alberts R	2017	107	3,159	Genetic association analysis identifies variants associated with disease progression in primary sclerosing cholangitis.
GCST90018565	Sakaue S	2021	85	166,529	A cross-population atlas of genetic associations for 220 human phenotypes.

Variant details and genetic-evidence tiers

Tier distribution (top 50 variants)

Tier	Variants
Tier 1: coding	1
Tier 2: splice/UTR	2
Tier 3: regulatory	0
Tier 4: intronic/intergenic	14

MAF distribution

Bucket	Variants
common (>=0.05)	14
low_freq (0.01-0.05)	1
rare (<0.01)	0
unknown	2

Functional consequences

Consequence	Count
intron_variant	8
intergenic_variant	4
unknown	2
5_prime_UTR_variant	1
missense_variant	1
3_prime_UTR_variant	1

Top variants

rsID	Chr	Pos	Alleles	MAF	Consequence	Gene	p-value	Tier
rs2187668	6	32638107	C>A,G,T	0.15	intron_variant	HLA-DQA1	2e-78	Tier 4: intronic/intergenic
rs6932730	6	31386405	T>C	0.05	intron_variant	MICA-AS1	9e-73	Tier 4: intronic/intergenic
rs1794514	6	32699696	G>A,C,T	0.05	intergenic_variant	HLA-DQB1 - MTCO3P1	6e-14	Tier 4: intronic/intergenic
DQA1*05:01							4e-11	Tier 4: intronic/intergenic
rs3891175	6	32666690	C>A,G,T	0.05	5_prime_UTR_variant	HLA-DQB1	5e-11	Tier 2: splice/UTR
rs72929257	2	204117920	C>T	0.05	intergenic_variant	ICOS - DSTNP5	3e-09	Tier 4: intronic/intergenic
rs6809477	3	63577606	C>A,T	0.05	intron_variant	SYNPR-AS1, SYNPR	5e-09	Tier 4: intronic/intergenic
rs56036302	6	32304379	A>T	0.2	intron_variant	TSBP1-AS1, TSBP1	7e-09	Tier 4: intronic/intergenic
rs564176274	6	32674248	C>T	0.05	intergenic_variant	HLA-DQB1 - MTCO3P1	9e-09	Tier 4: intronic/intergenic
rs3184504	12	111446804	T>A,C,G	0.43	missense_variant	ATXN2, SH2B3	8e-08	Tier 1: coding
rs11889341	2	191079016	C>T	0.05	intron_variant	STAT4	1e-07	Tier 4: intronic/intergenic
DQB1*02:01							2e-07	Tier 4: intronic/intergenic
rs9564997	13	73469604	C>A,G,T	0.05	intron_variant	LINC00393	3e-07	Tier 4: intronic/intergenic
rs7574865	2	191099907	T>A,G	0.05	intron_variant	STAT4	1e-06	Tier 4: intronic/intergenic
rs6000782	22	37532179	A>C	0.04	intron_variant	CARD10 - CDC42EP1	3e-06	Tier 4: intronic/intergenic
rs11084330	19	54333209	A>G	0.05	3_prime_UTR_variant	LILRA4	5e-06	Tier 2: splice/UTR
rs11117432	16	85985665	G>A	0.05	intergenic_variant	LINC02132 - LINC01082	6e-06	Tier 4: intronic/intergenic

Genes & proteins

Mendelian disease overlap and somatic drivers

GenCC: 0 · Orphanet: 9 · OMIM-shared: 0 · Dual-evidence (GWAS+Mendelian): 1

Dual-evidence genes (GWAS + Mendelian — highest-confidence targets)

Gene	HGNC	Evidence routes
SH2B3	SH2B3	GWAS, Orphanet

Orphanet rare-disease linkage (cohort genes)

Gene	Orphanet ID	Rare disease
SH2B3	Orphanet:3318	Essential thrombocythemia
SH2B3	Orphanet:391366	Growth retardation-mild developmental delay-chronic hepatitis syndrome
HLA-DQA1	Orphanet:391490	Adult-onset myasthenia gravis
HLA-DQA1	Orphanet:930	Idiopathic achalasia
HLA-DQB1	Orphanet:2073	Narcolepsy type 1
HLA-DQB1	Orphanet:477738	Pediatric multiple sclerosis
HLA-DQB1	Orphanet:703	Bullous pemphigoid
HLA-DQB1	Orphanet:83465	Narcolepsy type 2
HLA-DQB1	Orphanet:930	Idiopathic achalasia

Cohort genes → proteins

4 cohort genes, 4 distinct canonical proteins.

Evidence partition

Subset	Genes
gwas_only	4

Cohort genes (full)

Symbol	HGNC	Ensembl	UniProt	Name	Evidence
CARD10	HGNC:16422	ENSG00000100065	Q9BWT7	Caspase recruitment domain-containing protein 10	gwas
SH2B3	HGNC:29605	ENSG00000111252	Q9UQQ2	SH2B adapter protein 3	gwas
HLA-DQA1	HGNC:4942	ENSG00000196735	P01909	HLA class II histocompatibility antigen, DQ alpha 1 chain	gwas
HLA-DQB1	HGNC:4944	ENSG00000179344	P01920	HLA class II histocompatibility antigen, DQ beta 1 chain	gwas

Cohort function summary

Lead sentence per gene, UniProt-curated.

Symbol	Protein name	Function (lead sentence)
CARD10	Caspase recruitment domain-containing protein 10	Scaffold protein that plays an important role in mediating the activation of NF-kappa-B via BCL10 or EGFR.
SH2B3	SH2B adapter protein 3	Links T-cell receptor activation signal to phospholipase C-gamma-1, GRB2 and phosphatidylinositol 3-kinase.
HLA-DQA1	HLA class II histocompatibility antigen, DQ alpha 1 chain	Binds peptides derived from antigens that access the endocytic route of antigen presenting cells (APC) and presents them on the cell surface for recognition by the CD4 T-cells.
HLA-DQB1	HLA class II histocompatibility antigen, DQ beta 1 chain	Binds peptides derived from antigens that access the endocytic route of antigen presenting cells (APC) and presents them on the cell surface for recognition by the CD4 T-cells.

Protein-family classification

Druggable: 2 · Difficult: 1 · Unknown: 1 · Druggable fraction: 0.5

Family distribution

Cohort families vs a genome-wide background (hypergeometric, BH-FDR; fold = observed/expected). Counts kept; sorted by enrichment, so the catch-all Other/Unknown bucket no longer leads.

Family	Genes	Fold	FDR
Antibody/Immunoglobulin	2	14.6×	0.020
Scaffold/PPI	1	4.3×	0.318
Other/Unknown	1	0.5×	0.962

Per-gene assignment

Symbol	Family	Druggable?	InterPro (top 3)
CARD10	Other/Unknown	no	CARD, DEATH-like_dom_sf, P-loop_NTPase
SH2B3	Scaffold/PPI	no	SH2, PH_domain, PH-like_dom_sf
HLA-DQA1	Antibody/Immunoglobulin	yes	MHC_II_a_N, Ig/MHC_CS, Ig_C1-set
HLA-DQB1	Antibody/Immunoglobulin	yes	MHC_II_b_N, Ig/MHC_CS, Ig_C1-set

Expression context

Cohort genes with no expression data: 0.

4 cohort genes are a single-cell marker in ≥1 SCXA experiment.

Breadth distribution (Bgee present_calls)

Bucket	Genes
narrow (1-5 tissues)	0
moderate (6-20)	0
broad (>20)	4
unknown	0

Top tissues across cohort

Tissue	Cohort genes
monocyte	2
jejunal mucosa	1
pancreatic ductal cell	1
parotid gland	1
leukocyte	1
mononuclear cell	1
gall bladder	1
rectum	1
right lung	1
spleen	1
upper lobe of left lung	1

Per-gene tissue summary (top 30)

Symbol	Bgee breadth	FANTOM5 breadth	SCXA	Top tissues
CARD10	243	ubiquitous	marker	parotid gland, jejunal mucosa, pancreatic ductal cell
SH2B3	260	ubiquitous	marker	monocyte, mononuclear cell, leukocyte
HLA-DQA1	244	broad	marker	gall bladder, rectum, monocyte
HLA-DQB1	268	broad	marker	right lung, spleen, upper lobe of left lung

Protein interactions among cohort

Intra-cohort edges: 1.

Hub genes (top 10 by interactor count)

Symbol	Interactor count
CARD10	2,265
SH2B3	1,617
HLA-DQA1	196
HLA-DQB1	128

Intra-cohort edges

A	B	Sources
HLA-DQA1	HLA-DQB1	biogrid_interaction, intact

Structural data

PDB: 2 · AlphaFold-only: 2 · No structure: 0

Cohort genes with PDB structures (top 30)

Symbol	UniProt	PDB entries
HLA-DQA1	P01909	28
HLA-DQB1	P01920	10

AlphaFold-only cohort genes (top 30 by pLDDT)

Symbol	UniProt	pLDDT
CARD10	Q9BWT7	70.22
SH2B3	Q9UQQ2	63.45

Function

Pathway analysis

Distinct Reactome pathways touched by cohort: 17. Enrichment computed across 4 evidence-associated genes (3 with Reactome annotation).

Pathways by enrichment

Over-representation of cohort genes vs the genome-wide background (hypergeometric test, Benjamini-Hochberg FDR; fold = observed/expected over 3 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.

Pathway	Cohort genes	Fold	FDR	Sample cohort genes
Interferon gamma signaling	2	83.7×	0.003	HLA-DQA1, HLA-DQB1
Negative regulation of FLT3	1	237.9×	0.016	SH2B3
Translocation of ZAP-70 to Immunological synapse	1	211.5×	0.016	HLA-DQA1
Regulation of KIT signaling	1	200.3×	0.016	SH2B3
Phosphorylation of CD3 and TCR zeta chains	1	181.3×	0.016	HLA-DQA1
Co-inhibition by PD-1	1	173.0×	0.016	HLA-DQA1
Generation of second messenger molecules	1	115.3×	0.018	HLA-DQA1
FLT3 Signaling	1	115.3×	0.018	SH2B3
Signaling by SCF-KIT	1	82.8×	0.023	SH2B3
Downstream TCR signaling	1	42.8×	0.039	HLA-DQA1
MHC class II antigen presentation	1	29.7×	0.051	HLA-DQA1
Factors involved in megakaryocyte development and platelet production	1	22.1×	0.063	SH2B3
Signaling by Receptor Tyrosine Kinases	1	17.2×	0.074	SH2B3
Cytokine Signaling in Immune system	1	13.6×	0.087	SH2B3
Hemostasis	1	12.0×	0.092	SH2B3
Immune System	1	4.3×	0.227	SH2B3
Signal Transduction	1	3.4×	0.267	SH2B3

GO biological processes by enrichment

Over-representation of cohort genes vs the genome-wide background (hypergeometric test, Benjamini-Hochberg FDR; fold = observed/expected over 4 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.

GO term	Cohort genes	Fold	FDR	Sample cohort genes
peptide antigen assembly with MHC class II protein complex	2	526.6×	2e-04	HLA-DQA1, HLA-DQB1
antigen processing and presentation of exogenous peptide antigen via MHC class II	2	271.8×	3e-04	HLA-DQA1, HLA-DQB1
positive regulation of immune response	2	240.7×	3e-04	HLA-DQA1, HLA-DQB1
positive regulation of T cell activation	2	221.7×	3e-04	HLA-DQA1, HLA-DQB1
negative regulation of Kit signaling pathway	1	2106.5×	0.003	SH2B3
monocyte homeostasis	1	1404.3×	0.004	SH2B3
adaptive immune response	2	42.1×	0.004	HLA-DQA1, HLA-DQB1
negative regulation of receptor signaling pathway via STAT	1	842.6×	0.005	SH2B3
cellular response to interleukin-3	1	702.2×	0.005	SH2B3
negative regulation of response to cytokine stimulus	1	702.2×	0.005	SH2B3
negative regulation of chemokine-mediated signaling pathway	1	601.9×	0.005	SH2B3
thrombopoietin-mediated signaling pathway	1	526.6×	0.005	SH2B3
activation of NF-kappaB-inducing kinase activity	1	421.3×	0.006	CARD10
neutrophil homeostasis	1	383.0×	0.006	SH2B3
immune response	2	23.5×	0.006	HLA-DQA1, HLA-DQB1
negative regulation of platelet aggregation	1	351.1×	0.006	SH2B3
embryonic hemopoiesis	1	247.8×	0.007	SH2B3
negative regulation of cell migration involved in sprouting angiogenesis	1	247.8×	0.007	CARD10
cellular response to chemokine	1	247.8×	0.007	SH2B3
negative regulation of receptor signaling pathway via JAK-STAT	1	221.7×	0.008	SH2B3
hematopoietic stem cell differentiation	1	191.5×	0.008	SH2B3
megakaryocyte development	1	175.5×	0.009	SH2B3
erythrocyte development	1	131.7×	0.011	SH2B3
regulation of immune response	1	123.9×	0.011	CARD10
positive regulation of protein localization to nucleus	1	98.0×	0.014	CARD10
negative regulation of MAPK cascade	1	75.2×	0.017	SH2B3
humoral immune response	1	70.2×	0.018	HLA-DQB1
negative regulation of phosphatidylinositol 3-kinase/protein kinase B signal transduction	1	65.8×	0.018	SH2B3
T cell receptor signaling pathway	1	38.0×	0.031	HLA-DQB1
protein-containing complex assembly	1	28.5×	0.039	CARD10

Therapeutics

Drugs indicated for this disease

0 approved, 3 in late-stage (phase 3) trials. Disease-direct ChEMBL indications, not inferred from the associated-gene cohort below.

Drug	Development status
Cyclosporine	Phase 3 (in late-stage trials)
Paeoniflorin	Phase 3 (in late-stage trials)
Phosphatidylcholine	Phase 3 (in late-stage trials)

Earlier-phase candidates (phase 2, investigational — efficacy not yet established): Azathioprine, Budesonide, Cannabidiol, Ianalumab, Prednisone, Tacrolimus Anhydrous.

Drug target analysis

Approved (phase 4): 0 · Phase ≥3: 0 · Phased (≥1): 0 · Undrugged: 4

Druggability breadth: 1 of 4 evidence-associated genes (25%) have a ChEMBL target (buckets above are over the deeply-mined display cohort).

Top cohort targets by molecule count

Symbol	Molecules	Max phase
CARD10	0	0
SH2B3	0	0
HLA-DQA1	0	0
HLA-DQB1	0	0

Bioactivity and enzyme data

Enzyme cohort genes (≥1 EC): 0.

Cohort genes with ChEMBL bioactivity (full, sorted by assay count)

Symbol	Assays	Type breakdown
HLA-DQA1	2	Binding:2

Pharmacogenomics

Cohort genes with a PharmGKB record: 4; with CPIC/DPWG dosing guidelines: 0.

No cohort gene has a CPIC/DPWG genotype-guided dosing guideline (PharmGKB).

Chemical tractability of cohort targets

0 approved/phased compounds have measured bioactivity against a cohort gene (and aren’t yet in disease-level trials). This is a research / tractability signal, NOT a therapeutic recommendation — a bioactivity row often reflects off-target or screening binding (e.g. promiscuous kinase inhibitors against a cohort kinase), implying no disease mechanism.

Druggability pyramid

Cohort genes binned by druggability tier (high → low):

Tier	Definition	Genes	Symbols
A	Approved (phase 4 drug)	0
B	Phased (≥1) drug, not yet approved	0
C	Druggable family + PDB, no drug	2	HLA-DQA1, HLA-DQB1
D	Druggable family + AlphaFold only, no drug	0
E	Difficult family or no structure, no drug	2	CARD10, SH2B3

Undrugged target profiles

4 cohort genes are undrugged. Ranked by ‘starting-point quality’ (assay depth + drugged-partner adjacency).

Symbol	ChEMBL assays	Drugged partners (top 3)
CARD10	0	—
SH2B3	0	—
HLA-DQA1	2	—
HLA-DQB1	0	—

Clinical trials & evidence

Clinical trials

Clinical trials: 64.

Phase distribution (across all retrieved trials)

Phase	Trials
Not specified	39
PHASE2	9
PHASE4	5
PHASE1	4
PHASE2/PHASE3	3
PHASE3	2
PHASE1/PHASE2	1
EARLY_PHASE1	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT00687180	PHASE4	COMPLETED	Mycophenolate vs Azathioprin in Autoimmune Hepatitis
NCT02463331	PHASE4	COMPLETED	Possible Role of Chloroquine to Induce a Complete Remission in the Treatment of Autoimmune Hepatitis: a Randomized Trial
NCT02900443	PHASE4	UNKNOWN	Mycophenolate Mofetil Versus Azathioprine in Treatment Naive Autoimmune Hepatitis
NCT04933292	PHASE4	UNKNOWN	A Randomised Clinical Trial Assessing the Efficacy and Safety of Mycophenolate Mofetil Versus Azathioprine for Induction of Remission in Treatment Primary Biliary Cholangitis-Autoimmune Hepatitis Overlap Syndrome
NCT05221411	PHASE4	UNKNOWN	Tacrolimus Versus Mycophenolate for Autoimmune Hepatitis Patients With Incomplete Response on First Line Therapy
NCT06650124	PHASE2/PHASE3	NOT_YET_RECRUITING	Induction of Remission in Autoimmune Hepatitis With Azathioprine vs. MMF
NCT07598825	PHASE3	NOT_YET_RECRUITING	A Trial of Inebilizumab in Participants With Autoimmune Hepatitis
NCT00838214	PHASE2/PHASE3	COMPLETED	Budesonide 3x3mg/d Versus Prednisone in Active Autoimmune Hepatitis
NCT01170351	PHASE3	COMPLETED	Cyclosporine-A Versus Prednisolone for Induction of Remission in Auto-immune Hepatitis
NCT03217422	PHASE2/PHASE3	COMPLETED	ADCC Mediated B-Cell dEpletion and BAFF-R Blockade
NCT06381453	PHASE2	RECRUITING	Belimumab in Autoimmune Hepatitis
NCT00608894	PHASE2	TERMINATED	LCP-Tacro vs. Azathioprine for the Treatment of Autoimmune Hepatitis
NCT01661842	PHASE1/PHASE2	UNKNOWN	Umbilical Cord Mesenchymal Stem Cells for Patients With Autoimmune Hepatitis
NCT01988506	PHASE2	COMPLETED	Induction of Regulatory t Cells by Low Dose il2 in Autoimmune and Inflammatory Diseases
NCT02556372	PHASE2	COMPLETED	Liver Test Study of Using JKB-122 in Autoimmune Hepatitis (AIH) Patients Who Are Refractory or Intolerant to Current Therapies
NCT04129489	PHASE2	TERMINATED	A Phase 2a Study to Evaluate the Safety and Efficacy of Cannabidiol Only as Maintenance Therapy and Steroid Sparing in Patients With Stable Autoimmune Hepatitis
NCT04371718	PHASE2	UNKNOWN	Effect of JKB-122 on Prednisolone and Azathioprine Induced Remission in Autoimmune Hepatitis (AIH)
NCT04790916	PHASE2	TERMINATED	Effect of RO7049665 on the Time to Relapse Following Steroid Tapering in Participants With Autoimmune Hepatitis (AIH)
NCT05476900	PHASE2	COMPLETED	A Study to Evaluate the Efficacy and Safety of HR19042 Capsules in the Treatment of Autoimmune Hepatitis.
NCT05569759	PHASE2	COMPLETED	A Study of Zetomipzomib (KZR-616) in Patients With Autoimmune Hepatitis (PORTOLA)
NCT06455280	PHASE1	RECRUITING	A Study of SIPLIZUMAB in AILD and LT Patients
NCT02239562	PHASE1	COMPLETED	sPIF CLINICAL STUDY PROTOCOL IN AUTOIMMUNE HEPATITIS
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NCT04313205	PHASE1	UNKNOWN	A Pharmacokinetic Study of JKB-122 Tablets Compared to Capsule
NCT03842254	EARLY_PHASE1	COMPLETED	Use of Erythropoietin to Expand Regulatory T Cells in Autoimmune Liver Disease
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NCT04902807	Not specified	RECRUITING	Conception of a Diagnosis, Prognosis and Therapeutic Decision Tool for Patients With Autoimmunity and Inflammation
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Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
AZATHIOPRINE	4	5
MYCOPHENOLATE MOFETIL	4	3
CANNABIDIOL	4	1
CHLOROQUINE PHOSPHATE	4	1
INEBILIZUMAB	4	1
METHYLPREDNISOLONE	4	1
PREDNISONE	4	1
IANALUMAB	3	1
MELREDABLEUKIN ALFA	2	1
SIPLIZUMAB	2	1
ZETOMIPZOMIB	2	1
CHEMBL15720	0	1

Cohort genes: CARD10, SH2B3, HLA-DQA1, HLA-DQB1
Drugs: Azathioprine, Mycophenolate Mofetil, Cannabidiol, Chloroquine Phosphate, Inebilizumab, Methylprednisolone, Prednisone, Ianalumab