RARE DISEASERESEARCH ATLAS
Open navigation menu

ORPHA:829

Adult-onset Still disease

medium confidenceDisorder

Also known as: AOSD · Wissler-Fanconi syndrome

Publications

3,296

90.5th percentile

Trials

9

Interventional, condition-specific

Researchers

1,105

Distinct authors in sample

Gene link

—

Readiness

4/6

Stages with a signal

Clinical definition (Orphanet)

A rare inflammatory multisystem disorder characterized clinically by four cardinal signs: fever of unknown origin, arthralgia or arthritis, hyperleucocytosis, and typical skin rash.

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 (3)

Adult-Onset Still's Disease · adult-onset Still disease · adult-onset Still's 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.”

4/6 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

    3,296 matched papers (1,920 in last 10 years) Source

  3. Phenotype characterisedPresent

    59 HPO annotations (e.g. Fever; Weight loss; Abdominal pain) Source

  4. Animal modelNot found

    No Alliance genotype “model of” associations via Monarch for these Mondo IDs

  5. Orphan designationPresent

    4 FDA designations (3 FDA orphan-indication approvals) — e.g. tadekinig alfa Source

  6. Interventional trialPresent

    9 matched on ClinicalTrials.gov

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)

59

Associated phenotypes · MONDO:0019355

  • Fever
  • Weight loss
  • Abdominal pain
  • Pleuritis
  • Pleural effusion

Showing 5 of 59 — open Monarch for the full list.

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)

4

Designations · 3 with FDA orphan-indication approval

  • FDA tadekinig alfaAdult-Onset Still's Disease Still's disease Systemic juvenile idiopathic arthritis · 2017-07-03 · Not FDA Approved for Orphan Indication
  • FDA interleukin-1 receptor antagonist anakinraAdult-Onset Still's Disease Still's disease · 2015-09-15 · Not FDA Approved for Orphan Indication
  • FDA Interleukin-1 TrapAdult-Onset Still's Disease Still's disease Juvenile rheumatoid arthritis · 2005-04-04 · Not FDA Approved for Orphan Indication
  • FDA canakinumabAdult-Onset Still's Disease · 2017-07-31

Sources: FDA OOPD · EMA orphan designations

Open Targets candidates

14

Drugs / clinical candidates · MONDO_0019355

CTD chemicals (MyDisease.info)

3 associated chemicals. Therapeutic evidence is listed first when present — not a treatment recommendation.

  • Adrenal Cortex Hormones · therapeutic
  • Aspirin · therapeutic
  • Methotrexate · therapeutic

MyDisease.info · MONDO:0019355

Literature

Is anyone studying this?

3,296

3,296 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=3967) is 59.

3,296 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).

1,920 in the last 10 years · medium confidence · 90.5th percentile (publications denominator)

Phrase hits: 3,296 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,105

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
    Kaneko Y6 papers · 2026

    Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan.

    Papers in Europe PMC
  2. 02
    Feist E5 papers · 2026

    Department of Rheumatology and Clinical Immunology, Helios Fachklinik Vogelsang-Gommern Klinik für Rheumatologie, 39245 Gommern, Germany.

    Papers in Europe PMC
  3. 03
    Kawakami A5 papers · 2026

    Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, JPN.

    Papers in Europe PMC
  4. 04
    Rech J5 papers · 2026

    Department of Internal Medicine 3-Rheumatology and Immunology, Friedrich-Alexander University (FAU) Erlangen-Nürnberg and Universitätsklinikum Erlangen, 91054 Erlangen, Germany.

    Papers in Europe PMC
  5. 05
    Shi H5 papers · 2026

    Department of Rheumatology and Immunology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China.

    Papers in Europe PMC
  6. 06
    Asano T4 papers · 2026

    Department of Gastroenterology, Saitama Medical Center, Jichi Medical University, Japan.

    Papers in Europe PMC
  7. 07
    Chen X4 papers · 2026

    Department of Rheumatology and Immunology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China.

    Papers in Europe PMC
  8. 08
    Cheng X4 papers · 2026

    Department of Rheumatology and Immunology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China.

    Papers in Europe PMC
  9. 09
    Chi H4 papers · 2026

    Department of Rheumatology and Immunology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China.

    Papers in Europe PMC
  10. 10
    Fujita Y4 papers · 2026

    Department of Rheumatology, Fukushima Medical University School of Medicine, Fukushima, Japan.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

9

interventional trials for this specific condition

9 interventional trials matched this specific condition name; none in our sample are currently recruiting.

Data as of 11 September 2026

9 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 92th percentile).

medium confidence · 92th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

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

No currently recruiting studies in the matched set. Open the same search on ClinicalTrials.gov.

Observational and natural-history studies

7 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

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 2026-07-29

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)

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 Adult-onset Still disease — check Associations / patient organisations on that page.

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.

"Adult-onset Still disease" OR "Wissler-Fanconi syndrome" OR "Adult-Onset Still's Disease"

Run this search on Europe PMC

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

"Adult-onset Still disease" OR "Wissler-Fanconi syndrome" OR "Adult-Onset Still's 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: 9 interventional · 7 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

Synonyms dropped by stoplist: AOSD

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 2026-07-26T15:34:58.443Z