{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T21:48:52Z","timestamp":1777672132544,"version":"3.51.4"},"reference-count":14,"publisher":"SAGE Publications","license":[{"start":{"date-parts":[[2023,1,1]],"date-time":"2023-01-01T00:00:00Z","timestamp":1672531200000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Journal of Investigative Medicine High Impact Case Reports"],"published-print":{"date-parts":[[2023,1]]},"abstract":"<jats:p>Schnitzler\u2019s Syndrome (SS) is a rare late-onset acquired autoinflammatory disorder which consists of chronic urticaria associated with a monoclonal IgM-kappa gammopathy, arthralgias, skeletal hyperostosis, lymphadenopathy, and recurrent constitutional symptoms. The average age of diagnosis is 51 years with a slight male predominance with a male to female ratio of 1.6. Diagnosis of SS requires the presence of 2 major criteria including chronic urticaria and monoclonal IgM along with at least two of the following minor criteria: recurrent intermittent fevers, bone pain, arthralgias, elevated erythrocyte sedimentation rate (ESR), neutrophilic dermal infiltrate on skin biopsy, and leukocytosis or elevated C-reactive protein (CRP). Early diagnosis and clinical awareness are paramount in SS as it is associated with a 15-20% risk of lymphoproliferative malignancy. The median overall survival is 12.8 years. We present a case of a 39-year-old female with new onset urticaria associated with recurrent fevers and joint pain. Symptoms were refractory to steroids, and high dose antihistamines. Multi-disciplinary evaluation resulted in the ultimate diagnosis of Schnitzler\u2019s Syndrome. The patient was ultimately treated with canakinumab (Il-1 inhibitor), with near resolution of symptoms. This case demonstrates the importance of a broad differential diagnosis and maintaining a high clinical suspicion for rare diseases when presented with a complex form of an otherwise common condition.<\/jats:p>","DOI":"10.1177\/23247096231220480","type":"journal-article","created":{"date-parts":[[2023,12,22]],"date-time":"2023-12-22T02:33:10Z","timestamp":1703212390000},"update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":2,"title":["Clinical Presentation of Schnitzler\u2019s Syndrome: A Rare Autoimmune Disease"],"prefix":"10.1177","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3759-9523","authenticated-orcid":false,"given":"Vishal","family":"Patel","sequence":"first","affiliation":[{"name":"University of Florida Health, Gainesville, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Darshana","family":"Balasubramaniam","sequence":"additional","affiliation":[{"name":"University of Florida Health, Gainesville, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vanessa","family":"Cavero-Chavez","sequence":"additional","affiliation":[{"name":"University of Florida Health, Gainesville, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lyda","family":"Cuervo-Pardo","sequence":"additional","affiliation":[{"name":"University of Florida Health, Gainesville, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Catalina","family":"Sanchez-Alvarez","sequence":"additional","affiliation":[{"name":"University of Florida Health, Gainesville, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","published-online":{"date-parts":[[2023,12,21]]},"reference":[{"key":"bibr1-23247096231220480","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-5-38"},{"key":"bibr2-23247096231220480","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2013.084830"},{"key":"bibr3-23247096231220480","first-page":"7041576","volume":"2018","author":"Bashir M","year":"2018","journal-title":"Case Rep Rheumatol"},{"key":"bibr4-23247096231220480","doi-asserted-by":"publisher","DOI":"10.1016\/j.semarthrit.2007.04.001"},{"key":"bibr5-23247096231220480","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-019-00638-z"},{"key":"bibr6-23247096231220480","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2020.588322"},{"key":"bibr7-23247096231220480","doi-asserted-by":"publisher","DOI":"10.1016\/j.annder.2018.06.010"},{"key":"bibr8-23247096231220480","doi-asserted-by":"publisher","DOI":"10.1097\/MD.0b013e3181943f5e"},{"key":"bibr9-23247096231220480","doi-asserted-by":"publisher","DOI":"10.1007\/s11882-010-0160-9"},{"key":"bibr10-23247096231220480","doi-asserted-by":"publisher","DOI":"10.1007\/s10238-005-0071-6"},{"issue":"6","key":"bibr11-23247096231220480","first-page":"818","volume":"33","author":"Balci MA","year":"2015","journal-title":"Clin Exp Rheumatol"},{"key":"bibr12-23247096231220480","doi-asserted-by":"publisher","DOI":"10.1002\/art.1780250607"},{"key":"bibr13-23247096231220480","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2016.07.041"},{"key":"bibr14-23247096231220480","doi-asserted-by":"publisher","DOI":"10.1111\/j.1398-9995.2012.02843.x"}],"container-title":["Journal of Investigative Medicine High Impact Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/23247096231220480","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/full-xml\/10.1177\/23247096231220480","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/23247096231220480","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,29]],"date-time":"2026-04-29T08:03:50Z","timestamp":1777449830000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/10.1177\/23247096231220480"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,1]]},"references-count":14,"alternative-id":["10.1177\/23247096231220480"],"URL":"https:\/\/doi.org\/10.1177\/23247096231220480","relation":{},"ISSN":["2324-7096","2324-7096"],"issn-type":[{"value":"2324-7096","type":"print"},{"value":"2324-7096","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,1]]},"article-number":"23247096231220480"}}