ReviewArthritis & rheumatology (Hoboken, N.J.)2022
The Conundrum of Lung Disease and Drug Hypersensitivity-like Reactions in Systemic Juvenile Idiopathic Arthritis.
Review in Arthritis & rheumatology (Hoboken, N.J.), 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers, 1 of them a synthesis that pooled it.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
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
29 citing papers in PubMed, 1 synthesis or guideline pooled it, 60 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
- Benralizumab response in adult-onset Still's disease with eosinophilic complications and associated lung disease: DRESS or biologics-induced cytokine imbalance?EULAR rheumatology open · 2026Article
- Type I Interferon and Still Disease.Arthritis & rheumatology (Hoboken, N.J.) · 2026Article
- Baseline Clinical Features and Biomarker Profiles of the Childhood Arthritis and Rheumatology Research Alliance Systemic Juvenile Idiopathic Arthritis-Associated Lung Disease Cohort.Arthritis care & research · 2026Article
- Type I Interferon Signature is Associated With Lung Disease, Drug-Associated Immune Reactions, and Genetic Variation in Interferon-Linked Pathways in Still Disease.Arthritis & rheumatology (Hoboken, N.J.) · 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
- Efficacy of combined JAK1/2 inhibition and cyclosporine in paediatric-onset Still's disease with lung involvement: a case report.Pediatric rheumatology online journal · 2026Article
- Decoding pulmonary risk in childhood-onset still's disease: immunogenetic determinants and implications for risk stratification.Respiratory research · 2026Article
- Still's Disease and Autoinflammation: Positioning an Inflammatory Syndrome on the Autoinflammation-Autoimmunity Spectrum.Current rheumatology reports · 2026Review
- Impact of Interleukin-1 Blockade on the Development of Macrophage Activation Syndrome in Still Disease: Incidence and Diagnostic Validity of the EULAR/ACR/PRINTO 2016 MAS Classification Criteria.Arthritis & rheumatology (Hoboken, N.J.) · 2025Article
- Article
- 25 Years of Biologics for the Treatment of Pediatric Rheumatic Disease: Advances in Prognosis and Ongoing Challenges.Arthritis care & research · 2025Review
- Pharmacological and non-pharmacological treatments for refractory paediatric Still's disease: a scoping review.Rheumatology advances in practice · 2025Review
- Update on Biologic Therapy in Juvenile Idiopathic Arthritis: A Five-year Narrative Review.Biologics : targets & therapy · 2025Review
- Monitoring of Adverse Events and Safety in Autoinflammatory Diseases: Real-Life Data from the Eurofever Registry.Journal of clinical immunology · 2024Observational
- Disease Course, Treatments, and Outcomes of Children With Systemic Juvenile Idiopathic Arthritis-Associated Lung Disease.Arthritis care & research · 2024Article
- Single-cell RNA sequencing in juvenile idiopathic arthritis.Genes & diseases · 2024Review
- Recent advances and evolving concepts in Still's disease.Nature reviews. Rheumatology · 2024Review
- IL-1 Family Blockade in Cytokine Storm Syndromes.Advances in experimental medicine and biology · 2024Review
- Treatment of systemic juvenile idiopathic arthritis.Nature reviews. Rheumatology · 2023Review
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
2 authors at 2 institutions in 1 country.
Funding
Abstract
An unusual form of lung disease has begun to affect some children with systemic juvenile idiopathic arthritis (JIA), coincident with increasing utilization of interleukin-1 (IL-1) and IL-6 antagonists. Many children with systemic JIA-associated lung disease (SJIA-LD) have a history of clinical and laboratory features resembling drug reaction with eosinophilia and systemic symptoms (DRESS), a presentation now convincingly associated with HLA-DRB1*15. Treatment of DRESS typically requires drug discontinuation, a daunting prospect for clinicians and families who rely upon these agents. Here we review SJIA-LD and its associated DRESS-like phenotype. We suggest an alternative explanation, the cytokine plasticity hypothesis, proposing that IL-1 and IL-6 blockers modulate the milieu in which T cells develop, leading to a pathologic immune response triggered through exposure to common microbes, or to other exogenous or endogenous antigens, rather than to the drugs themselves. This hypothesis differs from DRESS in mechanism but also in clinical implications, predicting that control of pathogenic T cells could permit continued use of IL-1 and IL-6 antagonists in some individuals. The spectrum posed by these two hypotheses provides a conceptual framework that will guide investigation into the pathogenesis of SJIA-LD and may open up new therapeutic avenues for patients with systemic JIA.
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What OpenQuestion holds
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.