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ORPHA:85414

Systemic-onset juvenile idiopathic arthritis

medium confidenceDisorder

Also known as: Still disease · Systemic-onset JIA

Publications

3,163

90.2th percentile

Trials

8

Interventional, condition-specific

Researchers

1,122

Distinct authors in sample

Gene link

—

Readiness

4/6

Stages with a signal

Clinical definition (Orphanet)

A rare pediatric rheumatological disease characterized by the variable occurrence of chronic arthritis, intermittent high spiking fever, maculopapular rash during fever episodes, and/or , lymphadenopathy, and serositis.

How rare: 1-9 / 100 000 — about one to nine people per hundred thousand.

Orphanet entry

Cross-references

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

Additional Mondo synonyms (4)

SoJIA · sJIA · systemic polyarthritis · systemic-onset JIA

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

    3,163 matched papers (1,801 in last 10 years) Source

  3. Phenotype characterisedPresent

    22 HPO annotations (e.g. Skin rash; Arthritis; Joint swelling) Source

  4. Animal modelNot found

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

  5. Orphan designationPartial

    2 EMA designations (none yet with FDA orphan-indication approval) — e.g. givinostat Source

  6. Interventional trialPresent

    8 matched on ClinicalTrials.gov (1 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)

22

Associated phenotypes · MONDO:0019434

  • Skin rash
  • Arthritis
  • Joint swelling
  • Autoimmunity
  • Elevated erythrocyte sedimentation rate

Showing 5 of 22 — 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)

2

Designations · no FDA orphan-indication approval yet

  • EMA givinostatTreatment of systemic-onset juvenile idiopathic arthritis · 28/01/2010 · WithdrawnEMA designation
  • EMA recombinant human monoclonal antibody to human IL-1beta of the IgG1/K classTreatment of systemic-onset juvenile idiopathic arthritis · 05/02/2008 · WithdrawnEMA designation

Sources: FDA OOPD · EMA orphan designations

Open Targets candidates

11

Drugs / clinical candidates · MONDO_0019434

CTD chemicals (MyDisease.info)

No CTD chemical associations returned for this Mondo ID.

Literature

Is anyone studying this?

3,163

3,163 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.

3,163 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).

1,801 in the last 10 years · medium confidence · 90.2th percentile (publications denominator)

Phrase hits: 3,154 · MeSH hits: 9

Open Europe PMC search

Who's working on it?

1,122

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
    Horneff G5 papers · 2026

    German Center for Paediatric and Adolescent Rheumatology , Garmisch-Partenkirchen , Germany.

    Papers in Europe PMC
  2. 02
    Koné-Paut I5 papers · 2023

    Division of Pediatric Rheumatology, Bicêtre Hospital, APHP, University of Paris SUD, 78 rue du Général Leclerc, Le Kremlin-Bicêtre, Cedex, 94270, France.

    Papers in Europe PMC
  3. 03
    Belot A4 papers · 2025

    Pediatric Nephrology, Rheumatology, Dermatology Unit, Hôpital Femme Mère Enfant, Hospices Civils de Lyon, 69677 Bron, France.

    Papers in Europe PMC
  4. 04
    Chaussabel D4 papers · 2025

    Systems Immunology, Benaroya Research Institute, Seattle, WA, USA. dchaussabel@sidra.org.

    Papers in Europe PMC
  5. 05
    de Jager W4 papers · 2018

    Dept. of Pediatric Immunology, University Medical Center Utrecht, Utrecht, the Netherlands

    Papers in Europe PMC
  6. 06
    Rinchai D4 papers · 2025

    Research Branch, Sidra Medicine, Doha, Qatar. drinchai@sidra.org.

    Papers in Europe PMC
  7. 07
    Toufiq M4 papers · 2025

    Research Branch, Sidra Medicine, Doha, Qatar.

    Papers in Europe PMC
  8. 08
    Zhang Y4 papers · 2026

    Guangzhou Institute of Pediatrics, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, Guangzhou, Guangdong 510623, China.

    Papers in Europe PMC
  9. 09
    Abdwani R3 papers · 2025

    Department of Child Health, Sultan Qaboos University Hospital, University Medical City, Muscat, Oman.

    Papers in Europe PMC
  10. 10
    Aggarwal A3 papers · 2023

    Department of Clinical Immunology and Rheumatology, SGPGIMS, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raibareli Road, Lucknow, 226014, India. aa.amita@gmail.com.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

8

interventional trials for this specific condition

8 interventional trials matched this specific condition name; 1 currently recruiting in our sample. 172 trials are registered for juvenile idiopathic arthritis, the broader category — shown separately because they may or may not enrol this specific subtype.

Data as of 11 September 2026

8 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 91.5th percentile).

medium confidence · 91.5th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

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

Broader category: juvenile idiopathic arthritis

172

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

Observational and natural-history studies

13 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 41 · after dedupe 41 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 41 · 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 (41)

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 Systemic-onset juvenile idiopathic arthritis — 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.

"Systemic-onset juvenile idiopathic arthritis" OR "Still disease" OR "Systemic-onset JIA" OR "SoJIA" OR "systemic polyarthritis"

Run this search on Europe PMC

MeSH descriptor terms unioned into the query: Rheumatoid Arthritis, Systemic Juvenile

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

"Systemic-onset juvenile idiopathic arthritis" OR "Still disease" OR "Systemic-onset JIA" OR "SoJIA" OR "systemic polyarthritis" OR "Rheumatoid Arthritis, Systemic Juvenile"

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: 8 interventional · 13 observational · 0 expanded access. Only interventional studies enter the trial headline.

Parent-category trials query:

"juvenile idiopathic arthritis"

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

Run this search on ClinicalTrials.gov

Synonyms dropped by stoplist: sJIA

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 2026-07-27T03:00:24.695Z