ArticleJCI insight2017
Rilonacept maintains long-term inflammatory remission in patients with deficiency of the IL-1 receptor antagonist.
Article in JCI insight, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT01801449 (A Pilot Open-Label Study of Rilonacept), which is not on this map. Cited by 29 papers, 2 of them syntheses 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.
A Pilot Open-Label Study of Rilonacept (Arcalyst) in the Deficiency of the Interleukin-1 Receptor Antagonist (DIRA)
Who cites it
29 citing papers in PubMed, 2 syntheses or guidelines pooled it, 51 citations in OpenAlex.
- The 2021 EULAR/American College of Rheumatology Points to Consider for Diagnosis, Management and Monitoring of the Interleukin-1 Mediated Autoinflammatory Diseases: Cryopyrin-Associated Periodic Syndromes, Tumour Necrosis Factor Receptor-Associated Periodic Syndrome, Mevalonate Kinase Deficiency, and Deficiency of the Interleukin-1 Receptor Antagonist.Arthritis & rheumatology (Hoboken, N.J.) · 2022Pooled it
- Systematic Review of Safety and Efficacy of IL-1-Targeted Biologics in Treating Immune-Mediated Disorders.Frontiers in immunology · 2022Pooled it
- Advances in targeting IL-1 family cytokines for the treatment of inflammatory diseases.Nature reviews. Rheumatology · 2026Review
- Identification of the IL-1β/miRNA-326 axis as a key regulator of ferroptosis in spinal cord injury.BMC neuroscience · 2026Article
- Targeting IL-1β: a potential strategy for alleviating the immunopathology of aortic aneurysms.Frontiers in immunology · 2026Review
- Updated insights into the molecular networks for NLRP3 inflammasome activation.Cellular & molecular immunology · 2025Review
- Elevated Levels of IL-1Ra, IL-1β, and Oxidative Stress in COVID-19: Implications for Inflammatory Pathogenesis.Journal of clinical medicine · 2025Article
- Liposomes: An Emerging Strategy for the Effective Treatment of Rheumatoid Arthritis.Current rheumatology reviews · 2025Review
- Deficiency of interleukin-1 receptor antagonist: A systematic review.Archives of rheumatology · 2024Article
- An improved understanding of pediatric chronic nonbacterial osteomyelitis pathophysiology informs current and future treatment.Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research · 2024Review
- Disease flares with baricitinib dose reductions and development of flare criteria in patients with CANDLE/PRAAS.Annals of the rheumatic diseases · 2024Article
- Inflammatory turmoil within: an exploration of autoinflammatory disease genetic underpinnings, clinical presentations, and therapeutic approaches.Advances in rheumatology (London, England) · 2024Review
- Practical Approach to Diagnosis and Management of IL-1-Mediated Autoinflammatory Diseases (CAPS, TRAPS, MKD, and DIRA).Paediatric drugs · 2024Article
- The 4Pediatric rheumatology online journal · 2024Article
- Clinical Utility of Rilonacept for the Treatment of Recurrent Pericarditis: Design, Development, and Place in Therapy.Drug design, development and therapy · 2024Review
- Inflammasome Signaling, Thromboinflammation, and Venous Thromboembolism.JACC. Basic to translational science · 2023Review
- IL-1/MyD88-Dependent G-CSF and IL-6 Secretion Mediates Postburn Anemia.Journal of immunology (Baltimore, Md. : 1950) · 2023Article
- Juvenile idiopathic arthritis with systemic onset with inflammatory bone lesions: two case reports of patients successfully treated with canakinumab and experience gained from literature.Frontiers in pediatrics · 2023Article
- Pathophysiology, clinical manifestations and current management of IL-1 mediated monogenic systemic autoinflammatory diseases, a literature review.Pediatric rheumatology online journal · 2022Review
- Perspectives on anti-IL-1 inhibitors as potential therapeutic interventions for severe COVID-19.Cytokine · 2021Review
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
11 authors at 6 institutions in 2 countries.
Funding
Abstract
backgroundDeficiency of IL-1 receptor antagonist (DIRA) is a rare autoinflammatory disease that presents with life-threatening systemic inflammation, aseptic multifocal osteomyelitis, and pustulosis responsive to IL-1-blocking treatment. This study was performed (a) to investigate rilonacept, a long-acting IL-1 inhibitor, in maintaining anakinra-induced inflammatory remission in DIRA patients, (b) to determine doses needed to maintain remission, and (c) to evaluate the safety and pharmacokinetics of rilonacept in young children (<12 years).
methodsSix mutation-positive DIRA patients (children, ages 3-6 years), treated with daily anakinra, were enrolled into an open-label pilot study of subcutaneous rilonacept for 24 months. Clinical symptoms and inflammatory blood parameters were measured at all visits. A loading dose (4.4 mg/kg) was administered, followed by once weekly injections (2.2 mg/kg) for 12 months. Dose escalation (4.4 mg/kg) was allowed if inflammatory remission was not maintained. Subjects in remission at 12 months continued rilonacept for an additional 12 months.
resultsFive of six patients required dose escalation for findings of micropustules. Following dose escalation, all patients were in remission on weekly rilonacept administration, with stable laboratory parameters for the entire study period of 24 months. All children are growing at normal rates and have normal heights and weights. Quality of life improved while on rilonacept. No serious adverse events were reported.
conclusionRilonacept was found to maintain inflammatory remission in DIRA patients. The once weekly injection was well tolerated and correlated with increased quality of life, most likely related to the lack of daily injections.
trial registrationClinicalTrials.gov NCT01801449.
fundingNIH, NIAMS, and NIAID.
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Identifiers
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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.