SynthesisRMD open2020
Systematic literature review of efficacy/effectiveness and safety of current therapies for the treatment of cryopyrin-associated periodic syndrome, hyperimmunoglobulin D syndrome and tumour necrosis factor receptor-associated periodic syndrome.
Synthesis in RMD open, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.
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
22 citing papers in PubMed, 44 citations in OpenAlex.
- Biologic-biologic and biologic-JAK inhibitor combination therapy in refractory systemic autoinflammatory diseases.Seminars in arthritis and rheumatism · 2026Observational
- Non-decameric NLRP3 reveals a TGN/MTOC-distal pathway of inflammasome activation.Nature communications · 2026Article
- Long-term Effectiveness and Safety of Canakinumab in Patients with TRAPS: Analysis of the RELIANCE Non-Interventional Study.Rheumatology and therapy · 2026Article
- The expanding role of the NLRP3 inflammasome from periodic fevers to therapeutic targets.Nature immunology · 2025Review
- Review
- Long-Term Safety and Effectiveness of Canakinumab in Patients with MKD/HIDS: Interim Analysis of the RELIANCE Registry.Rheumatology and therapy · 2025Article
- Adult onset cryopyrin-associated periodic syndrome due to germline missense mutation inFrontiers in immunology · 2025Article
- Tocilizumab effectively reduces flares of hyperimmunoglobulin D syndrome in children: Three cases in China.Molecular genetics and metabolism reports · 2024Article
- Monitoring of Adverse Events and Safety in Autoinflammatory Diseases: Real-Life Data from the Eurofever Registry.Journal of clinical immunology · 2024Observational
- Tocilizumab for treating mevalonate kinase deficiency and TNF receptor-associated periodic syndrome: a case series and literature review.Pediatric rheumatology online journal · 2024Review
- A rare missense p.C125Y mutation in theFrontiers in genetics · 2024Article
- Dermatologic Manifestations of Noninflammasome-Mediated Autoinflammatory Diseases.JID innovations : skin science from molecules to population health · 2023Review
- Efficacy of Anakinra Treatment in two Moroccan Patients With Mevalonate Kinase Deficiency.Global pediatric health · 2023Article
- The Preferential Use of Anakinra in Various Settings of FMF: A Review Applied to an Updated Treatment-Related Perspective of the Disease.International journal of molecular sciences · 2022Review
- Chemical Modulation of Gasdermin-Mediated Pyroptosis and Therapeutic Potential.Journal of molecular biology · 2022Review
- Protective effect of anakinra on audiovestibular function in a murine model of endolymphatic hydrops.Frontiers in cellular neuroscience · 2022Article
- NLRP3 inflammasome and NLRP3-related autoinflammatory diseases: From cryopyrin function to targeted therapies.Frontiers in immunology · 2022Review
- [What is confirmed in the treatment of autoinflammatory fever diseases?]Der Internist · 2021Review
- Long-Term Efficacy of Anakinra in Cryopyrin-Associated Periodic Syndrome: Focus on Destructive Arthropathy.Journal of clinical immunology · 2021Article
- Reasons for canakinumab initiation among patients with periodic fever syndromes: a retrospective medical chart review from the United States.Pediatric rheumatology online journal · 2021Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
6 authors at 3 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectivesSeveral therapies are used for the treatment of rareautoinflammatory conditions like cryopyrin-associated periodic fever syndromes (CAPS), hyperimmunoglobulin Dsyndrome (HIDS)/mevalonate kinase deficiency (MKD) and tumour necrosis factor receptor-associated periodic syndrome (TRAPS). However, reviews reporting on treatment outcomes of these therapies are lacking.
methodsA systematic literature review was conducted using Embase, MEDLINE, MEDLINE-In Process and Cochrane databases to identify the randomised/non-randomised controlled trials (RCTs/non-RCTs) and real-world observational studies of CAPS, HIDS/MKD and TRAPS published as full-texts (January 2000-September 2017) or conference abstracts (January 2014-September 2017). Studies with data for ≥1 biologic were included. Studies with <5 patients were excluded.
resultsOf the 3 342 retrieved publications, 72 studies were included (CAPS, n=43; HIDS/MKD, n=9; TRAPS, n=7; studies with ≥2 cohorts, n=13). Most studies were full-text (n=56), published after 2010 (n=56) and real-world observational studies (n=58). Among included studies, four were RCTs (canakinumab, n=2 (CAPS, n=1; HIDS/MKD and TRAPS, n=1); rilonacept, n=1 (in CAPS); simvastatin, n=1 (in HIDS/MKD)). Canakinumab and anakinra were the most commonly used therapies for CAPS and HIDS/MKD, whereas etanercept, canakinumab and anakinra were the most common for TRAPS. The available evidence suggested the efficacy or effectiveness of canakinumab and anakinra in CAPS, HIDS/MKD and TRAPS, and of etanercept in TRAPS; asingle RCT demonstrated the efficacy of rilonacept in CAPS.
conclusionsCanakinumab, anakinra, etanercept and rilonacept were reported to be well tolerated; however, injection-site reactions were observed frequently with anakinra, rilonacept and etanercept. Data on the use of tocilizumab, infliximab and adalimumab in these conditions were limited; thus, further research is warranted.
Indexed as
Identifiers
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.