ArticleBiologics : targets & therapy2008
Long term management of patients with cryopyrin-associated periodic syndromes (CAPS): focus on rilonacept (IL-1 Trap).
Article in Biologics : targets & therapy, 2008. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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Who cites it
16 citing papers in PubMed, 39 citations in OpenAlex.
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- Increased expression of NLRP3 associated with elevated levels of HMGB1 in children with febrile seizures: a case-control study.BMC pediatrics · 2024Article
- Preventive Effects of Sinigrin Against the Memory Deterioration in the Pentylenetetrazole-Kindled Male Wistar Rats: Possible Modulation of NLRP3 Pathway.Neuromolecular medicine · 2022Article
- Activators of SIRT1 in the kidney and protective effects of SIRT1 during acute kidney injury (AKI) (effect of SIRT1 activators on acute kidney injury).Clinical and experimental nephrology · 2021Review
- The Overexpression of Sirtuin1 (SIRT1) Alleviated Lipopolysaccharide (LPS)-Induced Acute Kidney Injury (AKI) via Inhibiting the Activation of Nucleotide-Binding Oligomerization Domain-Like Receptors (NLR) Family Pyrin Domain Containing 3 (NLRP3) Inflammasome.Medical science monitor : international medical journal of experimental and clinical research · 2019Article
- The Role of NLRP3 and IL-1β in Refractory Epilepsy Brain Injury.Frontiers in neurology · 2019Article
- Effects of Anakinra on Health-Related Quality of Life in a Patient with 1129G>A/928G>A Mutations in MVK Gene and Heterozygosity for the Mutation 2107C>A in CIAS1 Gene.Frontiers in pediatrics · 2017Article
- Secondary amyloidosis in autoinflammatory diseases and the role of inflammation in renal damage.World journal of nephrology · 2016Review
- Systems Based Study of the Therapeutic Potential of Small Charged Molecules for the Inhibition of IL-1 Mediated Cartilage Degradation.PloS one · 2016Article
- Interleukin-1 as a common denominator from autoinflammatory to autoimmune disorders: premises, perils, and perspectives.Mediators of inflammation · 2015Review
- Treating inflammation by blocking interleukin-1 in humans.Seminars in immunology · 2013Review
- Characterization of NLRP3 variants in Japanese cryopyrin-associated periodic syndrome patients.Journal of clinical immunology · 2012Article
- Biologics in children's autoimmune disorders: efficacy and safety.European journal of pediatrics · 2011Review
- Muckle-Wells syndrome in an Indian family associated with NLRP3 mutation.Journal of postgraduate medicineArticle
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Authors and funding
3 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Cryopyrin-associated periodic syndromes (CAPS) are a group of inherited inflammatory disorders consisting of familial cold-induced autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS), and neonatal-onset multisystem inflammatory disease (NOMID; also known as chronic infantile neurologic, cutaneous, articular [CINCA] syndrome). These rare disorders are associated with heterozygous mutations in the NLRP3 (CIAS1) gene, which encodes the protein NALP3 or cryopyrin, and inflammation driven by excessive production of the cytokine interleukin-1beta (IL-1beta). Amyloidosis is a serious complication with 25% of MWS patients developing amyloidosis, with occasional fatal consequences, whilst up to 20% of CINCA/NOMID patients die from various complications, before reaching the early adulthood. In some CINCA/NOMID adult survivors amyloidosis can also occur. Prior to the discovery of the CIAS1 gene mutations and the advent of IL-1 targeted therapy, treatment was aimed at suppressing inflammation, with limited success. The selective blockade of IL-1beta, with anakinra (IL-1 receptor antagonist), not only provided supportive evidence for the role of IL-1beta in CAPS, but also demonstrated the efficacy of targeting IL-1beta for treatment of these conditions. In February, 2008, 'Orphan Drug' approval from the Food and Drug Administration (FDA) for rilonacept (IL-1 Trap/Arcalyst(), Regeneron Pharmaceuticals, Inc) was given for the treatment of two CAPS disorders, FCAS and MWS in adults and children 12 years and older, making rilonacept the first therapy approved for the treatment of CAPS.
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