ReviewAnnals of medicine2022
Refractory systemic onset juvenile idiopathic arthritis: current challenges and future perspectives.
Review in Annals of medicine, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 34 papers, 1 of them a synthesis that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
34 citing papers in PubMed, 1 synthesis or guideline pooled it, 44 citations in OpenAlex.
- EULAR/PReS recommendations for the diagnosis and management of Still's disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still's disease.Annals of the rheumatic diseases · 2024Guideline
- The role of ferroptosis in juvenile idiopathic arthritis: Causal inference and mediation by immune phenotypes.Medicine · 2026Article
- Distinct cytokine and chemokine alterations in bronchoalveolar fluid from patients with systemic juvenile idiopathic arthritis associated lung disease (SJIA-LD).Arthritis research & therapy · 2026Article
- Five-Year Outcomes of Systemic-Onset Juvenile Idiopathic Arthritis in India: Insights into Disease Course and Predictive Factors.European journal of rheumatology · 2026Article
- Case Report: Telitacicept in the treatment of refractory juvenile idiopathic arthritis: clinical experience from three cases.Frontiers in pediatrics · 2026Article
- Case Report: Treatment of refractory lung disease in systemic juvenile idiopathic arthritis with cyclophosphamide and rituximab combination therapy.Frontiers in immunology · 2026Article
- Evaluation of the real-world safety of Canakinumab in the treatment of systemic juvenile idiopathic arthritis in children aged 0-16: a comprehensive analysis based on FAERS data.Pediatric rheumatology online journal · 2025Article
- Emapalumab as a Life-Saving Therapy for Macrophage Activation Syndrome in Refractory Systemic Juvenile Idiopathic Arthritis with Trisomy 21.Turkish archives of pediatrics · 2025Article
- Long-term safety of canakinumab in patients with systemic juvenile idiopathic arthritis: 5-year results from the Childhood Arthritis and Rheumatology Research Alliance (CARRA) registry.Pediatric rheumatology online journal · 2025Article
- Associations of Lymphocyte Subpopulations, Related Cytokines, Clinical Phenotypes, and Outcomes in Systemic Juvenile Idiopathic Arthritis.ACR open rheumatology · 2025Article
- Rare coexistence of X-linked hyper immunoglobulin M syndrome and polyarticular juvenile idiopathic arthritis in a Chinese child: A case report.Joint diseases and related surgery · 2025Article
- Unmet needs and research gaps in Still's disease across ages: proceedings from a pediatric and adult joint expert panel.Pediatric rheumatology online journal · 2025Article
- Blood molecular subtypes to guide precision treatment strategies in systemic juvenile idiopathic arthritis.Arthritis research & therapy · 2025Article
- Health-Related Quality of Life in Juvenile Idiopathic Arthritis: A Systematic Review of Phase III Clinical Trials.Journal of clinical medicine · 2025Review
- Hypocomplementemia dynamics during tocilizumab therapy in systemic juvenile idiopathic arthritis: a retrospective longitudinal study.Frontiers in immunology · 2025Article
- Pharmacological and non-pharmacological treatments for refractory paediatric Still's disease: a scoping review.Rheumatology advances in practice · 2025Review
- Tofacitinib as an adjuvant treatment for pediatric Still's disease.Frontiers in pediatrics · 2025Article
- Tofacitinib treatment of systemic juvenile idiopathic arthritis: a case report and literature review.Frontiers in pediatrics · 2025Article
- Pan American League of Associations for Rheumatology treatment recommendations for systemic juvenile idiopathic arthritis.Rheumatology advances in practice · 2025Review
- Albumin and Gamma-Glutamyl Transferase as Biomarkers for Differentiating Systemic Juvenile Idiopathic Arthritis from Reactive Arthritis.Journal of inflammation research · 2025Article
Corrections and comments
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Authors and funding
4 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Systemic juvenile idiopathic arthritis (SJIA) is a rare disease with distinct features not seen in other categories of juvenile idiopathic arthritis. In recent years, advances in the understanding of disease immunopathogenesis have led to improved targeted therapies with significant improvement in patient outcomes. Despite these advances, there remain subsets of SJIA with refractory disease and severe disease-associated complications. This review highlights existing options for treatment of refractory SJIA and explores potential future therapeutics for refractory disease.Key Points:Despite targeted Interleukin IL-1 and IL-6 inhibitors a subset of SJIA remains refractory to therapy. About 1 in 7 SJIA patients will be refractory to targeted IL-1 or IL-6 therapy.There is no current agreed upon definition for refractory SJIA and we propose in this review that refractory SJIA is presence of active systemic or arthritic features despite treatment with anti-IL-1 or anti-IL-6 therapy or disease requiring glucocorticoids for control beyond 6 months.SJIA disease associated complications include presence of associated macrophage activation syndrome (MAS), interstitial lung disease (ILD) or amyloidosis and management of each differs.Refractory SJIA treatment options currently include additional conventional synthetic disease modifying anti-rheumatic drugs (csDMARDS), biologic (bDMARDS), combination biologic therapy, targeted synthetic (tsDMARDS) or other immunomodulatory therapies.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.