{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,9]],"date-time":"2026-07-09T19:57:34Z","timestamp":1783627054373,"version":"3.55.0"},"reference-count":46,"publisher":"Elsevier BV","issue":"1","license":[{"start":{"date-parts":[[2026,7,1]],"date-time":"2026-07-01T00:00:00Z","timestamp":1782864000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2026,7,1]],"date-time":"2026-07-01T00:00:00Z","timestamp":1782864000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2026,3,5]],"date-time":"2026-03-05T00:00:00Z","timestamp":1772668800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100003761","name":"Universitair Medisch Centrum Utrecht","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003761","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100016058","name":"Foundation Lymph&amp;Co","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100016058","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","jacionline.org","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Journal of Allergy and Clinical Immunology"],"published-print":{"date-parts":[[2026,7]]},"DOI":"10.1016\/j.jaci.2026.02.037","type":"journal-article","created":{"date-parts":[[2026,3,9]],"date-time":"2026-03-09T16:43:19Z","timestamp":1773074599000},"page":"253-268","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":1,"title":["Pyrin inflammasome activation triggers an IL-18\u2013driven IFN-\u03b3 response in mevalonate kinase deficiency"],"prefix":"10.1016","volume":"158","author":[{"given":"Niels S.","family":"van Heusden","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isa","family":"Cuijpers","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nils","family":"Meijer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daan","family":"Pieren","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angela","family":"Markovska","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cem","family":"Gabay","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charlotte","family":"Girard","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isabelle","family":"Kon\u00e9-Paut","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bas","family":"Vastert","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D\u00f6rte","family":"Hamann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Judith","family":"Jans","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erika","family":"Van Nieuwenhove","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joost","family":"Frenkel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2590-1692","authenticated-orcid":false,"given":"Marianne","family":"Boes","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.jaci.2026.02.037_bib1","doi-asserted-by":"crossref","first-page":"914","DOI":"10.1038\/ni.3457","article-title":"Pyrin inflammasome activation and RhoA signaling in the autoinflammatory diseases FMF and HIDS","volume":"17","author":"Park","year":"2016","journal-title":"Nat Immunol"},{"key":"10.1016\/j.jaci.2026.02.037_bib2","doi-asserted-by":"crossref","first-page":"5000","DOI":"10.1074\/jbc.M113.536920","article-title":"Defects in mitochondrial clearance predispose human monocytes to interleukin-1\u03b2 hypersecretion","volume":"289","author":"van der Burgh","year":"2014","journal-title":"J Biol Chem"},{"key":"10.1016\/j.jaci.2026.02.037_bib3","doi-asserted-by":"crossref","first-page":"1908","DOI":"10.1056\/NEJMoa1706314","article-title":"Canakinumab for the treatment of autoinflammatory recurrent fever syndromes","volume":"378","author":"De Benedetti","year":"2018","journal-title":"N Engl J Med"},{"key":"10.1016\/j.jaci.2026.02.037_bib4","doi-asserted-by":"crossref","first-page":"425","DOI":"10.3899\/jrheum.170684","article-title":"Efficacy of continuous interleukin 1 blockade in mevalonate kinase deficiency: a multicenter retrospective study in 13 adult patients and literature review","volume":"45","author":"Deshayes","year":"2018","journal-title":"J Rheumatol"},{"key":"10.1016\/j.jaci.2026.02.037_bib5","doi-asserted-by":"crossref","DOI":"10.15252\/emmm.201910547","article-title":"Pyrin dephosphorylation is sufficient to trigger inflammasome activation in familial Mediterranean fever patients","volume":"11","author":"Magnotti","year":"2019","journal-title":"EMBO Mol Med"},{"key":"10.1016\/j.jaci.2026.02.037_bib6","doi-asserted-by":"crossref","DOI":"10.1016\/j.celrep.2022.111472","article-title":"Steroid hormone catabolites activate the pyrin inflammasome through a non-canonical mechanism","volume":"41","author":"Magnotti","year":"2022","journal-title":"Cell Rep"},{"key":"10.1016\/j.jaci.2026.02.037_bib7","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1038\/s41419-023-05745-9","article-title":"Mutations in the B30.2 and the central helical scaffold domains of pyrin differentially affect inflammasome activation","volume":"14","author":"Chirita","year":"2023","journal-title":"Cell Death Dis"},{"key":"10.1016\/j.jaci.2026.02.037_bib8","doi-asserted-by":"crossref","first-page":"7148","DOI":"10.1073\/pnas.1103681108","article-title":"Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) is a disorder of innate immunity and Th1 activation responsive to IL-1 blockade","volume":"108","author":"Stojanov","year":"2011","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"1 pt 1","key":"10.1016\/j.jaci.2026.02.037_bib9","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1016\/S0091-6749(99)70518-X","article-title":"IL-18: a TH1-inducing, proinflammatory cytokine and new member of the IL-1 family","volume":"103","author":"Dinarello","year":"1999","journal-title":"J Allergy Clin Immunol"},{"key":"10.1016\/j.jaci.2026.02.037_bib10","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1016\/S1074-7613(00)80013-8","article-title":"Interleukin-18 binding protein: a novel modulator of the Th1 cytokine response","volume":"10","author":"Novick","year":"1999","journal-title":"Immunity"},{"key":"10.1016\/j.jaci.2026.02.037_bib11","doi-asserted-by":"crossref","first-page":"590","DOI":"10.1128\/iai.57.2.590-595.1989","article-title":"Endotoxin-induced serum factor that stimulates gamma interferon production","volume":"57","author":"Nakamura","year":"1989","journal-title":"Infect Immun"},{"key":"10.1016\/j.jaci.2026.02.037_bib12","doi-asserted-by":"crossref","DOI":"10.1016\/j.jbc.2022.101908","article-title":"Structural basis of human IL-18 sequestration by the decoy receptor IL-18 binding protein in inflammation and tumor immunity","volume":"298","author":"Detry","year":"2022","journal-title":"J Biol Chem"},{"key":"10.1016\/j.jaci.2026.02.037_bib13","doi-asserted-by":"crossref","first-page":"289","DOI":"10.3389\/fimmu.2013.00289","article-title":"Interleukin-18 and IL-18 binding protein","volume":"4","author":"Dinarello","year":"2013","journal-title":"Front Immunol"},{"key":"10.1016\/j.jaci.2026.02.037_bib14","doi-asserted-by":"crossref","first-page":"683","DOI":"10.1111\/j.1365-2362.1995.tb01986.x","article-title":"Interferon-gamma and urine neopterin in attacks of the hyperimmunoglobulinaemia D and periodic fever syndrome","volume":"25","author":"Drenth","year":"1995","journal-title":"Eur J Clin Invest"},{"key":"10.1016\/j.jaci.2026.02.037_bib15","doi-asserted-by":"crossref","first-page":"3586","DOI":"10.1182\/blood.V85.12.3586.bloodjournal85123586","article-title":"Cytokine activation during attacks of the hyperimmunoglobulinemia D and periodic fever syndrome","volume":"85","author":"Drenth","year":"1995","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2026.02.037_bib16","doi-asserted-by":"crossref","DOI":"10.3390\/metabo9010012","article-title":"Direct infusion based metabolomics identifies metabolic disease in patients\u2019 dried blood spots and plasma","volume":"9","author":"Haijes","year":"2019","journal-title":"Metabolites"},{"key":"10.1016\/j.jaci.2026.02.037_bib17","doi-asserted-by":"crossref","first-page":"114","DOI":"10.1016\/j.cyto.2017.10.021","article-title":"Effect of anticoagulants on 162 circulating immune related proteins in healthy subjects","volume":"106","author":"Scholman","year":"2018","journal-title":"Cytokine"},{"key":"10.1016\/j.jaci.2026.02.037_bib18","doi-asserted-by":"crossref","DOI":"10.3390\/ijerph18031170","article-title":"Gene expression analysis of mevalonate kinase deficiency affected children identifies molecular signatures related to hematopoiesis","volume":"18","author":"Pisanti","year":"2021","journal-title":"Int J Environ Res Public Health"},{"key":"10.1016\/j.jaci.2026.02.037_bib19","doi-asserted-by":"crossref","DOI":"10.3389\/fimmu.2024.1379220","article-title":"Mevalonate kinase\u2013deficient THP-1 cells show a disease-characteristic pro-inflammatory phenotype","volume":"15","author":"Politiek","year":"2024","journal-title":"Front Immunol"},{"key":"10.1016\/j.jaci.2026.02.037_bib20","doi-asserted-by":"crossref","first-page":"525","DOI":"10.1016\/j.trecan.2020.11.008","article-title":"Targeting the mevalonate pathway in cancer","volume":"7","author":"Juarez","year":"2021","journal-title":"Trends Cancer"},{"key":"10.1016\/j.jaci.2026.02.037_bib21","doi-asserted-by":"crossref","first-page":"922","DOI":"10.1038\/ni.3487","article-title":"Control of the innate immune response by the mevalonate pathway","volume":"17","author":"Akula","year":"2016","journal-title":"Nat Immunol"},{"key":"10.1016\/j.jaci.2026.02.037_bib22","doi-asserted-by":"crossref","first-page":"3975","DOI":"10.1038\/s41467-019-11606-x","article-title":"Protein prenylation restrains innate immunity by inhibiting Rac1 effector interactions","volume":"10","author":"Akula","year":"2019","journal-title":"Nat Commun"},{"key":"10.1016\/j.jaci.2026.02.037_bib23","doi-asserted-by":"crossref","first-page":"18134","DOI":"10.1074\/jbc.M200912200","article-title":"The structure of a binary complex between a mammalian mevalonate kinase and ATP: insights into the reaction mechanism and human inherited disease","volume":"277","author":"Fu","year":"2002","journal-title":"J Biol Chem"},{"key":"10.1016\/j.jaci.2026.02.037_bib24","first-page":"33","article-title":"Diagnostic value of urinary mevalonic acid excretion in patients with a clinical suspicion of mevalonate kinase deficiency (MKD)","volume":"27","author":"Jeyaratnam","year":"2016","journal-title":"JIMD Rep"},{"key":"10.1016\/j.jaci.2026.02.037_bib25","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1186\/s13007-023-01034-w","article-title":"A quantitative method to measure geranylgeranyl diphosphate (GGPP) and geranylgeranyl monophosphate (GGP) in tomato (Solanum lycopersicum) fruit","volume":"19","author":"Zita","year":"2023","journal-title":"Plant Methods"},{"key":"10.1016\/j.jaci.2026.02.037_bib26","doi-asserted-by":"crossref","first-page":"245","DOI":"10.1038\/s41392-024-01958-2","article-title":"Pyroptosis in health and disease: mechanisms, regulation and clinical perspective","volume":"9","author":"Liu","year":"2024","journal-title":"Signal Transduct Target Ther"},{"key":"10.1016\/j.jaci.2026.02.037_bib27","doi-asserted-by":"crossref","first-page":"1150","DOI":"10.3389\/fimmu.2020.01150","article-title":"Management of mevalonate kinase deficiency: a pediatric perspective","volume":"11","author":"Jeyaratnam","year":"2020","journal-title":"Front Immunol"},{"key":"10.1016\/j.jaci.2026.02.037_bib28","doi-asserted-by":"crossref","first-page":"26595","DOI":"10.1074\/jbc.272.42.26595","article-title":"Involvement of caspase-1 and caspase-3 in the production and processing of mature human interleukin 18 in monocytic THP.1 cells","volume":"272","author":"Akita","year":"1997","journal-title":"J Biol Chem"},{"key":"10.1016\/j.jaci.2026.02.037_bib29","doi-asserted-by":"crossref","DOI":"10.3390\/ijms20030649","article-title":"Interleukin-18 in health and disease","volume":"20","author":"Yasuda","year":"2019","journal-title":"Int J Mol Sci"},{"key":"10.1016\/j.jaci.2026.02.037_bib30","doi-asserted-by":"crossref","DOI":"10.1136\/rmdopen-2024-005388","article-title":"Elevated serum levels of interleukin-18 discriminate Still\u2019s disease from other autoinflammatory conditions: results from the European ImmunAID cohort","volume":"11","author":"Girard-Guyonvarc\u2019h","year":"2025","journal-title":"RMD Open"},{"key":"10.1016\/j.jaci.2026.02.037_bib31","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1038\/s41584-023-01053-w","article-title":"Biological and clinical roles of IL-18 in inflammatory diseases","volume":"20","author":"Landy","year":"2024","journal-title":"Nat Rev Rheumatol"},{"key":"10.1016\/j.jaci.2026.02.037_bib32","doi-asserted-by":"crossref","first-page":"2855","DOI":"10.1182\/blood-2010-07-294702","article-title":"I\u03baB\u03b6 augments IL-12\u2013 and IL-18\u2013mediated IFN-\u03b3 production in human NK cells","volume":"117","author":"Kannan","year":"2011","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2026.02.037_bib33","doi-asserted-by":"crossref","first-page":"212","DOI":"10.1111\/imcb.1032","article-title":"HLA-DR+ NK cells are mostly characterized by less mature phenotype and high functional activity","volume":"96","author":"Erokhina","year":"2018","journal-title":"Immunol Cell Biol"},{"key":"10.1016\/j.jaci.2026.02.037_bib34","doi-asserted-by":"crossref","DOI":"10.1371\/journal.ppat.1011502","article-title":"Parasite-induced IFN-\u03b3 regulates host defense via CD115 and mTOR-dependent mechanism of tissue-resident macrophage death","volume":"20","author":"Martin","year":"2024","journal-title":"PLoS Pathog"},{"key":"10.1016\/j.jaci.2026.02.037_bib35","doi-asserted-by":"crossref","first-page":"838","DOI":"10.1038\/ni.3205","article-title":"Interferon-\u03b3 regulates cellular metabolism and mRNA translation to potentiate macrophage activation","volume":"16","author":"Su","year":"2015","journal-title":"Nat Immunol"},{"key":"10.1016\/j.jaci.2026.02.037_bib36","doi-asserted-by":"crossref","first-page":"1451","DOI":"10.1182\/blood.V95.4.1451.004k52_1451_1455","article-title":"Hematopoietic-specific expression of MEFV, the gene mutated in familial Mediterranean fever, and subcellular localization of its corresponding protein, pyrin","volume":"95","author":"Tidow","year":"2000","journal-title":"Blood"},{"key":"10.1016\/j.jaci.2026.02.037_bib37","doi-asserted-by":"crossref","first-page":"3412","DOI":"10.1038\/s41467-020-17179-4","article-title":"Cholesterol metabolism drives regulatory B cell IL-10 through provision of geranylgeranyl pyrophosphate","volume":"11","author":"Bibby","year":"2020","journal-title":"Nat Commun"},{"key":"10.1016\/j.jaci.2026.02.037_bib38","doi-asserted-by":"crossref","first-page":"498","DOI":"10.1038\/s41467-019-08332-9","article-title":"The cholesterol biosynthesis pathway regulates IL-10 expression in human Th1 cells","volume":"10","author":"Perucha","year":"2019","journal-title":"Nat Commun"},{"key":"10.1016\/j.jaci.2026.02.037_bib39","doi-asserted-by":"crossref","first-page":"1140","DOI":"10.1038\/ng.3089","article-title":"An activating NLRC4 inflammasome mutation causes autoinflammation with recurrent macrophage activation syndrome","volume":"46","author":"Canna","year":"2014","journal-title":"Nat Genet"},{"issue":"suppl 1","key":"10.1016\/j.jaci.2026.02.037_bib40","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1186\/s12969-023-00867-y","article-title":"The 4th NextGen therapies of SJIA and MAS, part 4: it is time for IL-18 based trials in systemic juvenile idiopathic arthritis?","volume":"21","author":"Canna","year":"2024","journal-title":"Pediatr Rheumatol Online J"},{"key":"10.1016\/j.jaci.2026.02.037_bib41","doi-asserted-by":"crossref","first-page":"82","DOI":"10.1016\/j.ejim.2025.04.002","article-title":"Serum interleukin-18 levels are specifically elevated in auto-inflammatory diseases involving the pyrin inflammasome: a study on 516 patients","volume":"136","author":"Elhani","year":"2025","journal-title":"Eur J Intern Med"},{"key":"10.1016\/j.jaci.2026.02.037_bib42","doi-asserted-by":"crossref","first-page":"288","DOI":"10.1111\/cei.13620","article-title":"Lymphadenopathy at the crossroad between immunodeficiency and autoinflammation: an intriguing challenge","volume":"205","author":"Costagliola","year":"2021","journal-title":"Clin Exp Immunol"},{"key":"10.1016\/j.jaci.2026.02.037_bib43","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0237999","article-title":"Spectrum of clinical features and genetic variants in mevalonate kinase (MVK) gene of South Indian families suffering from hyperimmunoglobulin D syndrome","volume":"15","author":"Govindaraj","year":"2020","journal-title":"PLoS One"},{"key":"10.1016\/j.jaci.2026.02.037_bib44","doi-asserted-by":"crossref","first-page":"787","DOI":"10.1136\/ard-2023-225085","article-title":"Interrupting an IFN-\u03b3\u2013dependent feedback loop in the syndrome of pyogenic arthritis with pyoderma gangrenosum and acne","volume":"83","author":"Lee","year":"2024","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.jaci.2026.02.037_bib45","doi-asserted-by":"crossref","first-page":"857","DOI":"10.1136\/ard-2022-223739","article-title":"Efficacy and safety of emapalumab in macrophage activation syndrome","volume":"82","author":"De Benedetti","year":"2023","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.jaci.2026.02.037_bib46","doi-asserted-by":"crossref","first-page":"840","DOI":"10.1136\/annrheumdis-2017-212608","article-title":"Open-label, multicentre, dose-escalating phase II clinical trial on the safety and efficacy of tadekinig alfa (IL-18BP) in adult-onset Still\u2019s disease","volume":"77","author":"Gabay","year":"2018","journal-title":"Ann Rheum Dis"}],"container-title":["Journal of Allergy and Clinical Immunology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0091674926001752?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0091674926001752?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2026,7,5]],"date-time":"2026-07-05T00:07:56Z","timestamp":1783210076000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0091674926001752"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,7]]},"references-count":46,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2026,7]]}},"alternative-id":["S0091674926001752"],"URL":"https:\/\/doi.org\/10.1016\/j.jaci.2026.02.037","relation":{},"ISSN":["0091-6749"],"issn-type":[{"value":"0091-6749","type":"print"}],"subject":[],"published":{"date-parts":[[2026,7]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Pyrin inflammasome activation triggers an IL-18\u2013driven IFN-\u03b3 response in mevalonate kinase deficiency","name":"articletitle","label":"Article Title"},{"value":"Journal of Allergy and Clinical Immunology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.jaci.2026.02.037","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2026 The Authors. Published by Elsevier Inc. on behalf of the American Academy of Allergy, Asthma & Immunology.","name":"copyright","label":"Copyright"}]}}