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It is caused by specific mutations in the mevalonate kinase gene (MVK). No treatment specific to HIDS has been approved to date; however, nonsteroidal anti\u2010inflammatory drugs, steroids, colchicine, tumor necrosis factor\u2010\u03b1 inhibitors, and anti\u2010interleukin\u20101 treatments are used, based on case reports and observational studies. Herein, we report a case with recurrent fever and arthritis attacks who did not respond to anakinra and was successfully treated with canakinumab. Long\u2010term remission was achieved without any side effects with 300\u2009mg canakinumab treatment every 4\u2009weeks for 5\u2009years.<\/jats:p>","DOI":"10.1111\/1756-185x.14857","type":"journal-article","created":{"date-parts":[[2023,8,14]],"date-time":"2023-08-14T06:34:17Z","timestamp":1691994857000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Long\u2010term efficacy of canakinumab in hyperimmunoglobulin D syndrome"],"prefix":"10.1111","volume":"27","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2985-3723","authenticated-orcid":false,"given":"Ozlem","family":"Ozdemir\u00a0Isik","sequence":"first","affiliation":[{"name":"Division of Rheumatology, Department of Internal Medicine, School of Medicine Kocaeli University  Kocaeli Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5891-2032","authenticated-orcid":false,"given":"Duygu 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