ORPHA:563589
Seronegative autoimmune hepatitis
Also known as: Autoantibody-negative autoimmune hepatitis · Seronegative AIH
Publications
179
62.8th percentile
Trials
0
Interventional, condition-specific
Researchers
1,110
Distinct authors in sample
Gene link
—
Readiness
2/6
Stages with a signal
Clinical definition (Orphanet)
A form of autoimmune hepatitis characterized by the features of classic autoimmune hepatitis (i. e. clinical presentation as acute or chronic cryptogenic hepatitis, interface hepatitis on histological examination, elevated serum aspartate aminotransferase and alanine aminotransferase levels, hypergammaglobulinemia/elevated immunoglobulin G, therapeutic response to corticosteroids) in the absence of serum autoantibodies. Clinical manifestations include fatigue, malaise, arthralgia, jaundice, at later stages also signs of advanced chronic liver disease, such as spider nevi, caput medusae, , ascites, and palmar erythema. Presence of concurrent autoimmune diseases is frequently observed.
Cross-references
Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.
- MONDO:0035400
- UMLS:C5680121
Additional Mondo synonyms (1)
autoantibody-negative autoimmune hepatitis
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.
- Gene identifiedNot found
No GenCC disease–gene assertion in this build
- LiteraturePresent
179 matched papers (144 in last 10 years) Source
- Phenotype characterisedNot found
No HPO disease–phenotype associations via Monarch for these Mondo IDs
- Animal modelNot found
No Alliance genotype “model of” associations via Monarch for these Mondo IDs
- Orphan designationNot found
No FDA or EMA orphan-drug designation matched this disease via UMLS or preferred name Source
- 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)
None returned for this Mondo ID. That often means “not linked under this ID,” not “no clinical features.”
Animal models (Monarch / Alliance)
None returned for this Mondo ID. Empty here is not proof that no model organism work exists under another name or gene.
Monarch fetch 2026-07-29
Therapies
Designations, candidates, and chemicals
FDA OOPD and EMA orphan designations, Open Targets clinical candidates, and CTD chemical associations via MyDisease.info. These never change the interventional-trial headline.
Orphan designation (FDA · EMA)
No designation matched this disease via UMLS or preferred name on the FDA OOPD mirror or EMA orphan register. Absence here is not proof that none exists under another wording.
Open Targets candidates
No drugs or clinical candidates returned for this Mondo ID on Open Targets.
CTD chemicals (MyDisease.info)
No CTD chemical associations returned for this Mondo ID.
Literature
Is anyone studying this?
179
179 papers have ever been published on this condition (under this name). For scale, breast cancer has over 700,000. Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=3967) is 59.
179 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 59 (publications denominator n=3967).
144 in the last 10 years · high confidence · 62.8th percentile (publications denominator)
Phrase hits: 179 · MeSH hits: 0
Who's working on it?
1,110
Distinct author names in 179 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.
- 01Taubert R6 papers · 2025
Department of Gastroenterology, Hepatology and Endocrinology, Hannover Medical School, Hannover, Germany. taubert.richard@mh-hannover.de.
Papers in Europe PMC - 02Alam S5 papers · 2025
Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, New Delhi, 110070, India. seema_alam@hotmail.com.
Papers in Europe PMC - 03Czaja AJ5 papers · 2016
Department of Gastroenterology, Hacettepe University School of Ankara, Ankara, Turkey.
Papers in Europe PMC - 04Engel B5 papers · 2025
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 - 05Maggiore G5 papers · 2023
Pediatric Hepatology and Liver Transplantation, ISMETT-University of Pittsburgh Medical Center Italy, Palermo, Italy.
Papers in Europe PMC - 06Nastasio S5 papers · 2023
Division of Gastroenterology, Hepatology, and Nutrition, Harvard Medical School, Boston Children's Hospital, Boston, MA, United States.
Papers in Europe PMC - 07Sciveres M5 papers · 2023
Pediatric Hepatology and Liver Transplantation, ISMETT-University of Pittsburgh Medical Center Italy, Palermo, Italy.
Papers in Europe PMC - 08Dalekos GN4 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 - 09Jaeckel E4 papers · 2024
Department of Gastroenterology, Hepatology and Endocrinology, Hannover Medical School, Hannover, Germany.
Papers in Europe PMC - 10Manns MP4 papers · 2024
Department of Gastroenterology, Hepatology and Endocrinology, Hannover Medical School, Hannover, Germany.
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 11 September 2026 · last trial check 11 September 2026
No matched interventional trials. This is true for 77.2% of diseases in the trials denominator (5501 of 7126). Here are the researchers publishing on it.
high confidence · 38.6th 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
- NCT06650124·NOT YET RECRUITING·Induction of Remission in Autoimmune Hepatitis With Azathioprine vs. MMF
Conditions: Autoimmune Hepatitis·Matched via name phrase
- NCT07598825·NOT YET RECRUITING·A Trial of Inebilizumab in Participants With Autoimmune Hepatitis
Conditions: Autoimmune Hepatitis · AIH·Matched via name phrase
- NCT07118657·RECRUITING·Host-Diet-Gut Interaction Post Vegan Diet in Pediatric Autoimmune Hepatitis.
Conditions: Autoimmune Hepatitis·Matched via name phrase
- NCT05473403·NOT YET RECRUITING·Validation of a Prognostic Score for Steroid Therapy Response in Acute Severe Autoimmune Hepatitis
Conditions: Liver Failure, Acute · Hepatitis, Autoimmune · Organ Dysfunction Scores · Risk Factors·Matched via name phrase
- NCT06855667·NOT YET RECRUITING·Efficacy and Safety of Therapeutic Plasma Exchange vs Standard Medical Therapy in Severe Autoimmune Hepatitis.
Conditions: Autoimmune Hepatitis·Matched via name phrase
- NCT06381453·RECRUITING·Belimumab in Autoimmune Hepatitis
Conditions: Autoimmune Hepatitis·Matched via name phrase
- NCT06455280·RECRUITING·A Study of SIPLIZUMAB in AILD and LT Patients
Conditions: Autoimmune Liver Disease · Liver Transplant Disorder · Autoimmune Hepatitis · Primary Sclerosing Cholangitis·Matched via name phrase
- NCT06250309·RECRUITING·Mediterranean Diet Versus Western Diet on Fatigue in Autoimmune Hepatitis Patients
Conditions: Autoimmune Hepatitis·Matched via name phrase
Other registries (secondary)
Broader net from EU CTIS, ISRCTN, and ICTRP when available — deduped against ClinicalTrials.gov IDs already counted above. Dual-model LLM relevance gates what we keep. These rows are not added to the interventional headline.
raw 3 · after dedupe 3 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 3 · dropped 0 · fetched 2026-07-30
Source notes: ictrp: Error: ICTRP public search unavailable (WHO portal is SPA-only; SOAP needs partnership). Tried: https://apps.who.int/tri
No secondary-registry studies passed dual-model relevance for this condition name (after dedupe).
Uncertain / not reviewed (3)
- isrctn·ISRCTN15354495·No longer recruiting·Randomised trial optimising COVID-19 vaccination in patients with chronic health conditions and a poor response to standard vaccination
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN16993428·Recruiting·GenOMICC study - Looking at DNA of patients with severe illness and injury to find the genes that cause some people to become very unwell and be admitted to intensive care
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN70800019·No longer recruiting·Effects on tocilizumab drug therapy on fat tissue proteins in rheumatoid arthritis
skipped — LLM skipped (--skip-llm)
Where to find support
Condition-specific patient organisations, when Orphanet lists them, are on the disease’s Orphanet page. We also link umbrella groups that support undiagnosed and ultra-rare families.
Orphanet entry for Seronegative autoimmune hepatitis — check Associations / patient organisations on that page.
India — NPRD
Last verified 2026-07-26This 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.
"Seronegative autoimmune hepatitis" OR "Autoantibody-negative autoimmune hepatitis" OR "Seronegative AIH"
ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):
"Seronegative autoimmune hepatitis" OR "Autoantibody-negative autoimmune hepatitis" OR "Seronegative AIH"
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: ok — strategies attempted: phrase; 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 2026-07-27T18:21:22.986Z