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Systemic-onset juvenile idiopathic arthritis is marked by the severity of the extra-articular manifestations (fever, cutaneous eruptions) and by an equal sex ratio.
Biomarker and diagnostic research for systemic-onset juvenile idiopathic arthritis has been reported in the published literature.
No approved treatments are currently available for systemic-onset juvenile idiopathic arthritis. An additional 4 compounds hold orphan drug designation.
While no drugs are FDA-approved specifically for systemic-onset juvenile idiopathic arthritis, some of the following designated compounds may be used off-label in clinical practice. Treatment decisions should be made in consultation with a specialist familiar with this condition.
The following drugs have received orphan drug designation from the FDA for systemic-onset juvenile idiopathic arthritis. Orphan designation reflects regulatory interest and does not indicate approval for treatment.
Brand Name | Generic Name | Sponsor |
|---|
Estimated prevalence: 1-9 in 100,000 (Uncommon).
18 clinical trials registered, 8 recruiting. Interventions under study include other interventions, drug therapy, and biologic therapy. Pipeline includes 3 PHASE3, 2 PHASE2, 1 NA. Research is sponsored by a mix of industry and academic institutions.
NCT ID | Title | Phase | Sponsor | Status |
|---|---|---|---|---|
[NCT05609630](https://clinicaltrials.gov/study/NCT05609630) |
Data assembled from 5 of 12 sources · Last updated Sep 20, 2026, 8:41 AM UTC
European rare disease database
Genetic and Rare Diseases Info Center
Designated
Exclusivity End |
|---|
Designation Status |
|---|
Camoteskimab | Camoteskimab | Apollo Therapeutics Inc. | 2024 | — | Designated |
tadekinig alfa | tadekinig alfa | AB2 Bio Ltd | 2017 | — | Designated |
interleukin-1 receptor antagonist anakinra | interleukin-1 receptor antagonist anakinra | Swedish Orphan Biovitrum AB | 2015 | — | Designated |
Interleukin-1 Trap | Interleukin-1 Trap | Regeneron Pharmaceuticals, Inc. | 2005 | — | Designated |
Gene therapy approaches for systemic-onset juvenile idiopathic arthritis have been reported in the published literature.
18 trials found
Study of Oral Upadacitinib and Subcutaneous/Intravenous Tocilizumab to Evaluate Change in Disease Activity, Adverse Events and How Drug Moves Through the Body of Pediatric and Adolescent Participants With Active Systemic Juvenile Idiopathic Arthritis. |
PHASE3 |
AbbVie |
RECRUITING |
[NCT05925452](https://clinicaltrials.gov/study/NCT05925452) | To Evaluate the Efficacy and Safety of Genalumab for Injection in the Treatment of Active Systemic Juvenile Idiopathic Arthritis. | NA | Changchun GeneScience Pharmaceutical Co., Ltd. | RECRUITING |
[NCT02991469](https://clinicaltrials.gov/study/NCT02991469) | A Repeated Dose-finding Study of Sarilumab in Children and Adolescents With Systemic Juvenile Idiopathic Arthritis (SKYPS) | PHASE2 | Sanofi | RECRUITING |
[NCT07077304](https://clinicaltrials.gov/study/NCT07077304) | EACVI Study on Multimodality Cardiovascular Imaging of Inflammatory Cardiovascular Diseases | — | Assistance Publique - Hôpitaux de Paris | RECRUITING |
[NCT05927454](https://clinicaltrials.gov/study/NCT05927454) | Acostill ( RaDiCo Cohort) (RaDiCo Acostill) | — | Institut National de la Santé Et de la Recherche Médicale, France | RECRUITING |
75 publications have been identified in PubMed for systemic-onset juvenile idiopathic arthritis. Research spans Case Report / Case Series (34%), Epidemiology / Natural History (16%), and Review / Meta-Analysis (15%).
Research Type | Count | % of Total |
|---|---|---|
Patient case studies | 25 | 34% |
Disease patterns and progression | 12 | 16% |
Research summaries | 11 | 15% |
Laboratory research | 10 | 14% |
Other research | 6 | 8% |
Testing and diagnosis research | 6 | 8% |
Clinical study results | 3 | 4% |
New treatment approaches | 1 | 1% |
Lakhani HA (2026). [PMID: 41658735](https://pubmed.ncbi.nlm.nih.gov/41658735/). *Cureus*. [Review / Meta-Analysis]
Li J (2026). [PMID: 41692495](https://pubmed.ncbi.nlm.nih.gov/41692495/). *J Rheumatol*. [Other]
Mueller YM (2026). [PMID: 41728940](https://pubmed.ncbi.nlm.nih.gov/41728940/). *Arthritis Rheumatol*. [Basic Science / Preclinical]
Voulaz E (2026). [PMID: 41976374](https://pubmed.ncbi.nlm.nih.gov/41976374/). *Cancers (Basel)*. [Diagnostic / Biomarker]
Gomes C (2026). [PMID: 41853279](https://pubmed.ncbi.nlm.nih.gov/41853279/). *Front Immunol*. [Case Report / Case Series]
Balay-Dustrude E (2026). [PMID: 41033820](https://pubmed.ncbi.nlm.nih.gov/41033820/). *J Rheumatol*. [Review / Meta-Analysis]
Chen L (2026). [PMID: 41854276](https://pubmed.ncbi.nlm.nih.gov/41854276/). *Arthritis Rheumatol*. [Basic Science / Preclinical]
Botsa S (2026). [PMID: 41522543](https://pubmed.ncbi.nlm.nih.gov/41522543/). *Indian J Hematol Blood Transfus*. [Other]
Kobrin DM (2026). [PMID: 41397865](https://pubmed.ncbi.nlm.nih.gov/41397865/). *J Rheumatol*. [Basic Science / Preclinical]
Kieu Q (2026). [PMID: 41783506](https://pubmed.ncbi.nlm.nih.gov/41783506/). *SAGE Open Med Case Rep*. [Case Report / Case Series]
AI-curated news mentioning systemic-onset juvenile idiopathic arthritis
Updated Aug 24, 2026
A corrigendum has been issued for the study on serum-free IL-18 levels in systemic juvenile idiopathic arthritis, which utilized a novel detection method. This correction addresses previous findings published in Cytokine.
A recent study identifies distinct cytokine and chemokine alterations in bronchoalveolar fluid from patients with systemic juvenile idiopathic arthritis associated lung disease. These findings may enhance understanding of the disease's pathophysiology and inform future therapeutic strategies.
A recent literature review discusses the 2016 criteria for systemic juvenile idiopathic arthritis (sJIA) and macrophage activation syndrome (MAS), alongside a step-up therapeutic approach for Kawasaki disease-associated MAS. This combined cohort analysis provides insights into treatment strategies for these rare conditions.