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ORPHA:563581

Autoimmune hepatitis type 2

high confidenceSubtype of disorder

Also known as: AIH type 2

Query health: suspect — Only one of 2 strategies returned hits (phrase).

Publications

780

88.9th percentile

Trials

0

Interventional, condition-specific

Researchers

1,140

Distinct authors in sample

Gene link

—

Readiness

2/6

Stages with a signal

Clinical definition (Orphanet)

A form of autoimmune hepatitis characterized by clinical presentation as cryptogenic hepatitis, interface hepatitis on histological examination, elevated serum aminotransferase levels, hypergammaglobulinemia/elevated immunoglobulin G, and presence of circulating autoantibodies, specifically antibodies to liver kidney microsome type 1 (anti-LKM1) and anti-liver cytosol type 1 (anti-LC1) antibodies. The disease typically manifests in childhood or adolescence with an acute onset, often with acute liver failure. Long-term immunosuppression is usually required. Reported concurrent autoimmune diseases are autoimmune thyroiditis, diabetes mellitus, and vitiligo.

Orphanet entry

Cross-references

Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.

Additional Mondo synonyms (2)

autoimmune hepatitis type 2 · type 2 AIH

Research stages

Trial readiness signals

Where this condition sits on an open-data research pipeline — not how close a treatment is, and not medical advice. Empty stages often mean “not in these databases under this Mondo ID,” not “impossible.”

2/6 stages with a signal

No specific-condition interventional trial, but broader-category trials exist — discuss eligibility with a clinician.

  1. Gene identifiedNot found

    No GenCC disease–gene assertion in this build

  2. LiteraturePresent

    780 matched papers (429 in last 10 years) Source

  3. Phenotype characterisedNot checked

    Not yet enriched from Monarch / HPO

  4. Animal modelNot checked

    Not yet enriched from Monarch / Alliance

  5. Orphan designationNot checked

    FDA/EMA orphan-drug designation not enriched yet

  6. Interventional trialPartial

    None under the specific name; 41 for broader category autoimmune hepatitis

Biology

Genes and phenotypes

Gene–disease validity from GenCC, plus phenotypes and animal models joined from Monarch Initiative via Mondo ID — not a clinical diagnosis aid.

Do we know what causes it?

Not yet — the cause hasn't been pinned down in GenCC.

No strong gene–disease assertion joined for this Orphanet entity.

Phenotypes (Monarch / HPO)

Not enriched in this build — Monarch phenotype joins were not run for this record.

Animal models (Monarch / Alliance)

Not enriched in this build.

Literature

Is anyone studying this?

780

780 papers have been published on this condition. That is a real research literature — still far smaller than common diseases (breast cancer: over 700,000 papers). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=5449) is 41.

780 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 41 (publications denominator n=5449).

429 in the last 10 years · high confidence · 88.9th percentile (publications denominator)

Phrase hits: 780 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,140

Distinct author names in 200 sampled papers — named people below.

Who's working on it?

No interventional trial matched this name; these authors publish on it in the sampled literature — a practical starting point for contact.

  1. 01
    Mieli-Vergani G12 papers · 2025

    Paediatric Liver, GI & Nutrition Centre, MowatLabs, King's College Hospital, London, UK.

    Papers in Europe PMC
  2. 02
    Vergani D12 papers · 2025

    Institute of Liver Studies King's College Hospital, London, UK.

    Papers in Europe PMC
  3. 03
    Liu Y7 papers · 2026

    Department of Otorhinolaryngology-Head and Neck Surgery, Deparment of Allergy, The First Affiliated Hospital of Sun Yat-sen University, Sun Yat-sen University, Guangzhou, China.

    Papers in Europe PMC
  4. 04
    Dalekos GN5 papers · 2026

    Department of Medicine and Research Laboratory of Internal Medicine, Expertise Center of Greece in Autoimmune Liver Diseases, General University Hospital of Larissa, Larissa, Greece (George N. Dalekos, Eirini I. Rigopoulou).

    Papers in Europe PMC
  5. 05
    Liberal R5 papers · 2025

    Rodrigo Liberal, Charlotte R Grant, Institute of Liver Studies, King's College London School of Medicine at King's College Hospital, London SE 9RS, United Kingdom.

    Papers in Europe PMC
  6. 06
    Terziroli Beretta-Piccoli B5 papers · 2025

    Epatocentro Ticino, Lugano, Switzerland.

    Papers in Europe PMC
  7. 07
    Christen U4 papers · 2022

    Pharmazentrum Frankfurt/ZAFES, Goethe University Hospital, Theodor-Stern Kai 7, 60590 Frankfurt am Main, Germany. christen@med.uni-frankfurt.de.

    Papers in Europe PMC
  8. 08
    Engel B4 papers · 2026

    Department of Gastroenterology, Hepatology, Infectious Diseases and Endocrinology, Hannover Medical School, Carl-Neuberg-Straße 1, 30625, Hannover, Germany. Engel.Bastian@mh-hannover.de.

    Papers in Europe PMC
  9. 09
    Jaeckel E4 papers · 2026

    Department of Gastroenterology, Hepatology, Infectious Diseases and Endocrinology, Hannover Medical School, Carl-Neuberg-Straße 1, 30625, Hannover, Germany.

    Papers in Europe PMC
  10. 10
    Ma Y4 papers · 2025

    Institute of Liver Studies, King's College London School of Medicine at King's College Hospital, Denmark Hill, London SE5 9RS, UK.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

0

interventional trials for this specific condition

No interventional trial testing a treatment matched this specific condition name on ClinicalTrials.gov (observational studies and pan-disease registries are listed separately when present). 41 trials are registered for autoimmune hepatitis, the broader category — shown separately because they may or may not enrol this specific subtype.

Data as of 27 July 2026

No matched interventional trials. This is true for 73% of diseases in the trials denominator (5114 of 7003). Here are the researchers publishing on it.

high confidence · 36.5th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

No interventional trial testing a treatment was found for this specific condition name on ClinicalTrials.gov.

41 interventional trials matched autoimmune hepatitis, the broader category — listed below. Those studies are not counted in the condition-specific total.

Broader category: autoimmune hepatitis

41

Interventional trials for the parent category, exclusive of NCT IDs already counted above. Eligibility for this subtype is not guaranteed.

Worth raising with a clinician. How we count trials.

Recruiting under the broader category

Where to find support

We do not yet link condition-specific patient organisations. These umbrella groups support undiagnosed and ultra-rare families:

India — NPRD

Last verified 2026-07-26

This ORPHAcode is not on our curated NPRD list (direct or Mondo-parent match). That does not decide clinical eligibility; families in India should ask a notified Centre of Excellence about current coverage.

Hand-curated for this project. ORPHAcode mappings are best-effort and may be incomplete or imprecise for umbrella categories. Parent (Mondo) matches mean the policy lists a broader category — confirm eligibility with a Centre of Excellence. Financial entitlements summarised from public policy statements and may change. This is not official government guidance.

How we counted this

Europe PMC query (preferred label + any corrected label + Orphanet and Mondo exact synonyms, stoplisted; unioned with resolved MeSH labels when available). UMLS / OMIM / NCIT cross-references are stored on the overview but are not added to the query string.

"Autoimmune hepatitis type 2" OR "AIH type 2" OR "type 2 AIH"

Run this search on Europe PMC

ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):

"Autoimmune hepatitis type 2" OR "AIH type 2" OR "type 2 AIH" OR "autoimmune disorder of endocrine system" OR "autoimmune disorder of exocrine system" OR "autoimmune disorder of gastrointestinal tract"

Recall-expansion terms: autoimmune disorder of endocrine system, autoimmune disorder of exocrine system, autoimmune disorder of gastrointestinal tract

Study-type breakdown: 0 interventional · 0 observational · 0 expanded access. Only interventional studies enter the trial headline.

Parent-category trials query:

"autoimmune hepatitis"

Query health: suspect — strategies attempted: phrase, recall-expansion; with hits: phrase

Run this search on ClinicalTrials.gov

Confidence reasoning

  • Preferred label is multi-word and distinctive
  • No synonyms dropped by stoplist
  • No label/synonym collisions with other diseases in this corpus

Ingested 2026-07-27T18:21:13.974Z