ArticleRheumatology (Oxford, England)2020
IL-18 as a biomarker linking systemic juvenile idiopathic arthritis and macrophage activation syndrome.
Article in Rheumatology (Oxford, England), 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 67 papers, 5 of them syntheses that pooled it.
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Who cites it
67 citing papers in PubMed, 5 syntheses or guidelines pooled it, 109 citations in OpenAlex.
- Risk factors for macrophage activation syndrome in systemic juvenile idiopathic arthritis: a systematic review and meta-analysis.Frontiers in pediatrics · 2025Pooled it
- Immunopathology of and potential therapeutics for secondary hemophagocytic lymphohistiocytosis/macrophage activation syndrome: a translational perspective.Experimental & molecular medicine · 2024Pooled it
- Interleukin-18 Gene Polymorphisms and Rheumatoid Arthritis Susceptibility: An Umbrella Review of Meta-Analyses.Journal of immunology research · 2024Pooled it
- Update in Hyperferritinemic Syndromes: Recognition and Management - A Scoping Review.The Brown journal of hospital medicine · 2022Pooled it
- The role of interleukin-18 in the diagnosis and monitoring of hemophagocytic lymphohistiocytosis/macrophage activation syndrome - a systematic review.Clinical and experimental immunology · 2021Pooled it
- Complexities in the Diagnostic Evaluation and Treatment of Infant Hemophagocytic Lymphohistiocytosis.Hematology reports · 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
- Cytokine-based strategies to improve prognostic enrichment of pediatric ARDS.Critical care (London, England) · 2026Observational
- Speaking the same language: international cross-validation of emerging biomarkers for juvenile idiopathic arthritis and Still's Disease.Pediatric rheumatology online journal · 2026Article
- Pathogenic and genetic landscape of Still's disease across ages, with new insights into age-related IL-18 patterns.Arthritis research & therapy · 2026Review
- The Roles of Interleukin-18 in Inflammation and Autoimmune Disease.Clinical reviews in allergy & immunology · 2026Review
- Cytokine storm.Nature reviews. Disease primers · 2026Review
- Associations of Lymphocyte Subpopulations, Related Cytokines, Clinical Phenotypes, and Outcomes in Systemic Juvenile Idiopathic Arthritis.ACR open rheumatology · 2025Article
- Promising Targeted Therapies for Hemophagocytic Lymphohistiocytosis: A Translational Perspective Based on Immunopathology.Clinical reviews in allergy & immunology · 2025Review
- 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
- Long-term efficacy of MAS825, a bispecific anti-IL1β and IL-18 monoclonal antibody, in two patients with systemic JIA and recurrent episodes of macrophage activation syndrome.Rheumatology (Oxford, England) · 2025Article
- Lack of HLH in FMF.Pediatric rheumatology online journal · 2025Article
- Advancing multidisciplinary management of pediatric hyperinflammatory disorders.Frontiers in pediatrics · 2025Review
- IL-18 and IL-18BP: A Unique Dyad in Health and Disease.International journal of molecular sciences · 2024Review
- Biomarker Changes in Response to Tofacitinib Treatment in Patients With Polyarticular-Course Juvenile Idiopathic Arthritis.Arthritis care & research · 2024Article
7 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
9 authors at 4 institutions in 2 countries.
Funding
Abstract
objectivesSystemic juvenile idiopathic arthritis (sJIA) is a childhood arthritis with features of autoinflammation and high risk of macrophage activation syndrome (MAS). IL-18 has been shown to have key roles in sJIA and MAS. We aimed to examine IL-18 levels in sJIA in relation to disease activity and history of MAS and other disease biomarkers namely S100 proteins and CXCL9.
methodsTotal IL-18, CXCL9 and S100 proteins levels were determined in 40 sJIA patients, and IL-18 levels were compared between patients with regards to disease activity, history of MAS, and other biomarkers.
resultsTotal IL-18 levels were significantly higher in patients with active sJIA (median 16 499 pg/ml; interquartile range (IQR) 4816-61 839), and remained persistently elevated even in the majority of patients with inactive disease (1164 pg/ml; IQR 587-3444). Patients with history of MAS had significantly higher IL-18 levels (13 380 pg/ml; IQR 4212-62 628) as compared with those without MAS history (956.5 pg/ml; IQR 276.3-4262.5). Total IL-18 performed well with area under the curve of 0.8145 and 0.84 in predicting disease activity and history of MAS, respectively. We observed moderate correlation between IL-18 and CXCL9 (R = 0.56), S100A8/A9 (R = 0.47) and S100A12 (R = 0.46). The correlation was stronger for ferritin (R = 0.74) and overall for those with active disease.
conclusionTotal IL-18 levels were elevated in the majority of sJIA patients regardless of clinical features, but were higher in patients with active disease and history of MAS. Change in IL-18 may reflect increased disease activity or development of MAS.
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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.