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ORPHA:117

Behçet disease

medium confidenceDisorder

Publications

15,701

97.8th percentile

Trials

54

Interventional, condition-specific

Researchers

1,155

Distinct authors in sample

Gene link

—

Readiness

2/6

Stages with a signal

Clinical definition (Orphanet)

A rare, chronic, relapsing, multisystemic vasculitis characterized by mucocutaneous lesions, as well as articular, vascular, ocular and central nervous system manifestations.

How rare: 1-9 / 1 000 000 — roughly one to nine people per million. In a city the size of Kolkata, perhaps a few dozen.

Orphanet entry

Cross-references

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

Additional Mondo synonyms (9)

Bechet syndrome · Behcet disease · Behcet syndrome · Behcet's syndrome · Behçet syndrome · Behçet's syndrome · Behçet-Adamantiades syndrome · Morbus Behçet's syndrome · silk road disease

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

    15,701 matched papers (5,653 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

    54 matched on ClinicalTrials.gov (14 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?

15,701

15,701 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.

15,701 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).

5,653 in the last 10 years · medium confidence · 97.8th percentile (publications denominator)

Phrase hits: 15,701 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,155

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
    Hatemi G20 papers · 2026

    Department of Internal Medicine, Division of Rheumatology, Cerrahpaşa Medical School, Istanbul University-Cerrahpaşa, Istanbul, Türkiye.

    Papers in Europe PMC
  2. 02
    Seyahi E15 papers · 2026

    Division of Rheumatology, Department of Internal Medicine, Istanbul University-Cerrahpasa, Cerrahpasa Medical Faculty, Istanbul, Turkey.

    Papers in Europe PMC
  3. 03
    Esatoglu SN12 papers · 2026

    Department of Internal Medicine, Division of Rheumatology, Cerrahpaşa Medical School, Istanbul University-Cerrahpaşa, Istanbul, Türkiye.

    Papers in Europe PMC
  4. 04
    Talarico R11 papers · 2026

    Rheumatology Unit, Azienda Ospedaliero-Universitaria Pisana, Pisa, Italy. sara.talarico76@gmail.com.

    Papers in Europe PMC
  5. 05
    Wang X10 papers · 2026

    Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.

    Papers in Europe PMC
  6. 06
    Ozguler Y9 papers · 2026

    Department of Internal Medicine, Division of Rheumatology, Cerrahpaşa Medical School, Istanbul University-Cerrahpaşa, Istanbul, Türkiye.

    Papers in Europe PMC
  7. 07
    Emmi G8 papers · 2026

    Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy; Centre for Inflammatory Diseases, Monash University Department of Medicine, Monash Medical Centre, Melbourne, VIC, Australia. Electronic address: giacomo.emmi@unifi.it.

    Papers in Europe PMC
  8. 08
    Di Cianni F7 papers · 2026

    Rheumatology Unit, Azienda Ospedaliero-Universitaria Pisana, Pisa, and Department of Medical Biotechnologies, University of Siena, Italy.

    Papers in Europe PMC
  9. 09
    Liu J7 papers · 2026

    Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.

    Papers in Europe PMC
  10. 10
    Saadoun D7 papers · 2026

    From Sorbonne Universités Assistance Publique-Hôpitaux de Paris (AP-HP), Groupe Hospitalier Pitié-Salpêtrière, Département de Médecine Interne et Immunologie Clinique, Centre National de Références Maladies Autoimmunes Systémiques Rares, Centre National de Références Maladies Autoinflammatoires et Amylose Inflammatoire, Institut Hospitalo-Universitaire FOReSIGHT, Inflammation-Immunopathology-Biotherapy Department, Clinical Investigation Center in Biotherapy, and INSERM 959, Groupe Hospitalier Pitié-Salpêtrière, AP-HP (D.S., P.C.), and Sorbonne Universités AP-HP, Centre National de Références Maladies Rares en Ophtalmologie, Institut Hospitalo-Universitaire FOReSIGHT, Groupe Hospitalier Pitié-Salpêtrière, Département d'Ophtalmologie (B.B.) - all in Paris.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

54

interventional trials for this specific condition

54 interventional trials matched this specific condition name; 14 currently recruiting in our sample.

Data as of 27 July 2026

54 interventional trials — more than 73% of diseases in the trials denominator have none at all (5114 of 7003; this disease is at the 97.2th percentile).

medium confidence · 97.2th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

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

Observational and natural-history studies

35 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

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.

"Behçet disease" OR "Bechet syndrome" OR "Behcet disease" OR "Behcet syndrome" OR "Behcet's syndrome" OR "Behçet syndrome" OR "Behçet's syndrome" OR "Behçet-Adamantiades syndrome" OR "Morbus Behçet's syndrome" OR "silk road disease"

Run this search on Europe PMC

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

"Behçet disease" OR "Bechet syndrome" OR "Behcet disease" OR "Behcet syndrome" OR "Behcet's syndrome" OR "Behçet syndrome" OR "Behçet's syndrome" OR "Behçet-Adamantiades syndrome" OR "Morbus Behçet's syndrome" OR "silk road disease"

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: 54 interventional · 35 observational · 0 expanded access. Only interventional studies enter the trial headline.

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

Run this search on ClinicalTrials.gov

Confidence reasoning

  • Preferred label is short or not clearly distinctive
  • No synonyms dropped by stoplist
  • No label/synonym collisions with other diseases in this corpus

Ingested 2026-07-26T12:29:32.655Z