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ORPHA:186

Primary biliary cholangitis

medium confidenceDisorder

Also known as: Hanot syndrome · PBC · Primary biliary cirrhosis

Publications

30,190

99th percentile

Trials

142

Interventional, condition-specific

Researchers

1,097

Distinct authors in sample

Gene link

—

Readiness

2/6

Stages with a signal

Clinical definition (Orphanet)

A rare autoimmune cholestatic liver disease characterized by autoimmune mediated damage of small intrahepatic bile ducts leading to cholestasis, fibrosis, and potential cirrhosis.

How rare: 1-5 / 10 000 — about one to five people per ten thousand (still uncommon, but less ultra-rare).

Orphanet entry

Cross-references

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

Additional Mondo synonyms (4)

chronic non-suppurative destructive cholangitis · chronic nonsuppurative destructive cholangitis · primary Bilary cirrhosis (PBC) · primary biliary cirrhosis

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

An interventional trial matched this condition name on ClinicalTrials.gov — see trials below.

  1. Gene identifiedNot found

    No GenCC disease–gene assertion in this build

  2. LiteraturePresent

    30,190 matched papers (15,718 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 trialPresent

    142 matched on ClinicalTrials.gov (21 recruiting in sample)

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?

30,190

30,190 papers — among the better-studied rare conditions, though still a fraction of common-disease literature (breast cancer: over 700,000). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=5449) is 41.

30,190 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).

15,718 in the last 10 years · medium confidence · 99th percentile (publications denominator)

Phrase hits: 30,190 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,097

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

Who's working on it?

People publishing on this condition (sampled Europe PMC records). Affiliation is the most recent found in that sample.

  1. 01
    Wang L7 papers · 2026

    Department of Infectious Disease and Liver Disease & Outpatient, Follow-Up Center, Nanjing Key Laboratory of Hepatology, the Second Hospital of Nanjing, Affiliated to Nanjing University of Chinese Medicine, Nanjing, China.

    Papers in Europe PMC
  2. 02
    Wang Y7 papers · 2026

    Department of Pediatrics, The First Affiliated Hospital, Jiangxi Medical College, Nanchang University, Nanchang, Jiangxi, China.

    Papers in Europe PMC
  3. 03
    Levy C6 papers · 2026

    Schiff Center for Liver Diseases, University of Miami Miller School of Medicine, Miami, Florida, USA.

    Papers in Europe PMC
  4. 04
    Zhang Y6 papers · 2026

    Department of Infectious Disease and Liver Disease & Outpatient, Follow-Up Center, Nanjing Key Laboratory of Hepatology, the Second Hospital of Nanjing, Affiliated to Nanjing University of Chinese Medicine, Nanjing, China.

    Papers in Europe PMC
  5. 05
    Bowlus CL5 papers · 2026

    Division of Gastroenterology and Hepatology, University of California Davis School of Medicine, Sacramento, California, USA.

    Papers in Europe PMC
  6. 06
    Han Y5 papers · 2026

    Department of Gastroenterology and Hepatology, Laboratory for Clinical Medicine, Beijing You'an Hospital, Capital Medical University, Beijing, China.

    Papers in Europe PMC
  7. 07
    Hirschfield GM5 papers · 2026

    The Autoimmune and Rare Liver Disease Programme, Division of Gastroenterology and Hepatology, Toronto General Hospital, Toronto, Ontario, Canada.

    Papers in Europe PMC
  8. 08
    Li Y5 papers · 2026

    Department of Translational Medicine Center, Hangzhou Normal University Affiliated Hospital, Hangzhou, Zhejiang, China; Institute of Hepatology and Metabolic Diseases, Hangzhou Normal University, Hangzhou, Zhejiang, China; Department of School of Life Sciences, Zhejiang University of Traditional Chinese Medicine, Hangzhou, Zhejiang, 310053, China.

    Papers in Europe PMC
  9. 09
    Liu Y5 papers · 2026

    Second Department of Liver Disease Center, Beijing You 'an Hospital, Capital Medical University, Beijing 100069, China. Electronic address: yanmin130@ccmu.edu.cn.

    Papers in Europe PMC
  10. 10
    Tanaka A5 papers · 2026

    Department of Medicine, Teikyo University School of Medicine, Tokyo, Japan.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

142

interventional trials for this specific condition

142 interventional trials matched this specific condition name; 21 currently recruiting in our sample.

Data as of 27 July 2026

142 interventional trials — more than 73% of diseases in the trials denominator have none at all (5114 of 7003; this disease is at the 98.9th percentile).

medium confidence · 98.9th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

142 interventional trials matched after quoted-phrase search and title/condition post-filter.

Observational and natural-history studies

44 observational studies match this condition. These do not test a treatment and are not counted in the interventional-trial headline, but they are genuine research: natural-history work often defines the endpoints needed for a future rare-disease trial, and families may be able to enroll.

Recruiting or not-yet-recruiting

Open the complete matched search on ClinicalTrials.gov

General rare disease registries you may be eligible for

These studies enroll across many rare conditions. They are not counted as evidence that anyone is studying this specific disease.

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.

"Primary biliary cholangitis" OR "Hanot syndrome" OR "Primary biliary cirrhosis" OR "chronic non-suppurative destructive cholangitis" OR "chronic nonsuppurative destructive cholangitis" OR "primary Bilary cirrhosis (PBC)"

Run this search on Europe PMC

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

"Primary biliary cholangitis" OR "Hanot syndrome" OR "Primary biliary cirrhosis" OR "chronic non-suppurative destructive cholangitis" OR "chronic nonsuppurative destructive cholangitis" OR "primary Bilary cirrhosis (PBC)"

Interventional trials matched via: phrase (mesh = registered under a MeSH descriptor no name phrase would catch; recall-expansion = gene / selected parent terms used only for trials).

Study-type breakdown: 142 interventional · 44 observational · 1 expanded access. Only interventional studies enter the trial headline.

Query health: ok — strategies attempted: phrase; with hits: phrase

Run this search on ClinicalTrials.gov

Synonyms dropped by stoplist: PBC

Confidence reasoning

  • Preferred label is multi-word and distinctive
  • 1 synonym(s) dropped by stoplist (may under-count)
  • No label/synonym collisions with other diseases in this corpus

Ingested 2026-07-26T12:49:33.250Z