{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,21]],"date-time":"2026-08-21T12:23:09Z","timestamp":1787314989311,"version":"build-2736575974"},"reference-count":16,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2025,6,5]],"date-time":"2025-06-05T00:00:00Z","timestamp":1749081600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"abstract":"<jats:p>Cryopyrin-associated periodic syndrome is a rare autoinflammatory syndrome caused by a dysregulation of cytokine signaling pathways, particularly of the interleukin-1\u03b2 production. Symptoms typical appear early in life and include recurrent fever, arthralgia, and cutaneous rash, often accompanied by systemic manifestations and progressive end-organ damage resulting from chronic inflammation. Interleukin- targeted therapy constitutes the basis for treatment, and can lead to complete resolution of symptoms. We report a case of a 66-year-old man with cryopyrin-associated periodic syndrome related to V198M mutation, successfully treated with anakinra, an interleukin- 1 inhibitor frequently used in rheumatoid arthritis.<\/jats:p>","DOI":"10.3389\/fimmu.2025.1591234","type":"journal-article","created":{"date-parts":[[2025,6,5]],"date-time":"2025-06-05T01:28:22Z","timestamp":1749086902000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":2,"title":["Use of anakinra in cryopyrin-associated periodic syndromes: case report and review of literature"],"prefix":"10.3389","volume":"16","author":[{"given":"Marta","family":"Salas S\u00e1nchez","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Garc\u00eda-Mart\u00ednez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H\u00e9ctor","family":"Balastegui Mart\u00edn","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mar\u00eda Esther","family":"Dur\u00e1n-Garc\u00eda","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Lavilla Olleros","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2025,6,5]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-019-00638-z","article-title":"CAPS and NLRP3","volume":"39","author":"Booshehri","year":"2019","journal-title":"J Clin Immunol"},{"key":"B2","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1111\/j.1365-2230.2007.02540.x","article-title":"Cryopyrin-associated periodic syndromes and autoinflammation","volume":"33","author":"Shinkai","year":"2008","journal-title":"Clin Exp Dermatol"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.2165\/11595040-000000000-00000","article-title":"Cryopyrin-associated periodic syndromes: diagnosis and management","volume":"14","author":"Miyamae","year":"2012","journal-title":"Paediatr Drugs"},{"key":"B4","doi-asserted-by":"publisher","first-page":"12","DOI":"10.1007\/s11882-010-0160-9","article-title":"Cryopyrin-associated periodic syndrome: an update on diagnosis and treatment response","volume":"11","author":"Yu","year":"2011","journal-title":"Curr Allergy Asthma Rep"},{"key":"B5","doi-asserted-by":"publisher","first-page":"596","DOI":"10.1016\/j.berh.2017.12.001","article-title":"Periodic fever syndromes","volume":"31","author":"Lachmann","year":"2017","journal-title":"Best Pract Res Clin Rheumatol"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.3390\/jcm10010128","article-title":"Diagnosis and management of the cryopyrin-associated periodic syndromes (CAPS): what do we know today","volume":"10","author":"Welzel","year":"2021","journal-title":"J Clin Med"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1002\/art.40208","article-title":"Clinical and molecular phenotypes of low-penetrance variants of NLRP3: diagnostic and therapeutic challenges","volume":"69","author":"Kuemmerle-Deschner","year":"2017","journal-title":"Arthritis Rheumatol"},{"key":"B8","doi-asserted-by":"publisher","first-page":"R30","DOI":"10.1186\/ar4171","article-title":"Clinical characteristics in subjects with NLRP3 V198M diagnosed at a single UK center and a review of the literature","volume":"15","author":"Rowczenio","year":"2013","journal-title":"Arthritis Res Ther"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1212\/NXI.0000000000000109","article-title":"Expanding spectrum of neurologic manifestations in patients with NLRP3 low-penetrance mutations","volume":"2","author":"Schuh","year":"2015","journal-title":"Neurol Neuroimmunol Neuroinflamm"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.1007\/s10067-013-2481-2","article-title":"Renal involvement in secondary amyloidosis of Muckle-Wells syndrome: marked improvement of renal function and reduction of proteinuria after therapy with human anti-interleukin-1\u03b2 monoclonal antibody canakinumab","volume":"34","author":"Scarpioni","year":"2015","journal-title":"Clin Rheumatol"},{"key":"B11","doi-asserted-by":"publisher","first-page":"R34","DOI":"10.1186\/ar3266","article-title":"Canakinumab (ACZ885, a fully human IgG1 anti-IL-1\u03b2 mAb) induces sustained remission in pediatric patients with cryopyrin-associated periodic syndrome (CAPS)","volume":"13","author":"Kuemmerle-Deschner","year":"2011","journal-title":"Arthritis Res Ther"},{"key":"B12","doi-asserted-by":"publisher","DOI":"10.1186\/1546-0096-9-S1-P9","article-title":"A refractory heterogeneous Cryopyrin-Associated Periodic Syndrome (CAPS) phenotype related to V198M mutation responds to canakinumab - 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