{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,2,21]],"date-time":"2025-02-21T23:16:40Z","timestamp":1740179800779,"version":"3.37.3"},"reference-count":21,"publisher":"Oxford University Press (OUP)","issue":"1","license":[{"start":{"date-parts":[[2022,5,27]],"date-time":"2022-05-27T00:00:00Z","timestamp":1653609600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"funder":[{"name":"Instituto de Salud Carlos III through the project","award":["PI21\/01352"],"award-info":[{"award-number":["PI21\/01352"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,1,3]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n               <jats:p>Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome, a polygenic or multifactorial condition, is the most frequent autoinflammatory disease in children. There is increasing evidence that some patients may have a disease onset during adulthood. With regard to PFAPA syndrome treatment, single medium-to-high doses of glucocorticoids during flares constitute the therapy of choice in children and adults, colchicine may be useful in some patients, and tonsillectomy has been reported of utility mainly in paediatric patients. Interleukin-1 (IL-1) blockers have been sporadically used with good response in glucocorticoid-resistant cases. We report a patient with an adult onset of glucocorticoid-resistant PFAPA syndrome and inconsistent response to colchicine and anakinra, who later achieved a complete and sustained response to canakinumab. Although canakinumab seems to be a good therapeutic option in paediatric and adult patients with refractory PFAPA syndrome, the best anti-IL-1 agent and the sequence of administration have to be still determined in well-designed clinical trials.<\/jats:p>","DOI":"10.1093\/mrcr\/rxac043","type":"journal-article","created":{"date-parts":[[2022,5,31]],"date-time":"2022-05-31T20:02:19Z","timestamp":1654027339000},"page":"276-279","source":"Crossref","is-referenced-by-count":4,"title":["Efficacy of canakinumab in a patient with adult-onset glucocorticoid-resistant periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome"],"prefix":"10.1093","volume":"7","author":[{"given":"Bernardo","family":"Sope\u00f1a","sequence":"first","affiliation":[{"name":"Department of Internal Medicine, Complejo Hospitalario Universitario de Santiago, University of Santiago de Compostela , Santiago de Compostela, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Olga","family":"Ara\u00fajo","sequence":"additional","affiliation":[{"name":"Vasculitis Research Unit and Clinical Unit of Autoinflammatory Diseases, Department of Autoimmune Diseases, Hospital Clinic of Barcelona, Institut d\u2019Investigacions Biom\u00e8diques August Pi i Sunyer (IDIBAPS), University of Barcelona , Barcelona, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mayka","family":"Freire","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Complejo Hospitalario Universitario de Santiago, University of Santiago de Compostela , Santiago de Compostela, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luc\u00eda","family":"Barrera-L\u00f3pez","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Complejo Hospitalario Universitario de Santiago, University of Santiago de Compostela , Santiago de Compostela, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2357-2015","authenticated-orcid":false,"given":"Jos\u00e9","family":"Hern\u00e1ndez-Rodr\u00edguez","sequence":"additional","affiliation":[{"name":"Vasculitis Research Unit and Clinical Unit of Autoinflammatory Diseases, Department of Autoimmune Diseases, Hospital Clinic of Barcelona, Institut d\u2019Investigacions Biom\u00e8diques August Pi i Sunyer (IDIBAPS), University of Barcelona , Barcelona, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2022,5,27]]},"reference":[{"key":"2023010315521156200_R1","doi-asserted-by":"crossref","first-page":"1125","DOI":"10.1093\/rheumatology\/ket460","article-title":"International periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis syndrome cohort: description of distinct phenotypes in 301 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