{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,10]],"date-time":"2026-06-10T22:27:02Z","timestamp":1781130422834,"version":"3.54.1"},"reference-count":65,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2025,1,28]],"date-time":"2025-01-28T00:00:00Z","timestamp":1738022400000},"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>Schnitzler syndrome (SchS) is a rare acquired systemic autoinflammatory disorder, characterized by chronic urticarial rash and immunoglobulin M (IgM) monoclonal gammopathy. Anti-interleukin-1 (IL-1) therapies have been shown to be more effective in managing the clinical symptoms of SchS compared to anti-IL-6 therapies. In this case report, we present a male patient with urticarial rash, fever, and arthralgia. Laboratory tests identified the presence of IgM\u03ba monoclonal protein, and the absence of IL-1\u03b2 in serum. Whole exome sequencing (WES) did not reveal any pathological variants associated with monogenic autoinflammatory diseases or the <jats:italic>MYD88<\/jats:italic> L265P mutation. He met the diagnostic criteria for SchS and was treated with bortezomib, leading to a significant improvement in clinical symptoms and a decline in IgM\u03ba monoclonal protein levels. The patient tolerated the treatment well. This case suggests that bortezomib may be considered as a potential treatment option for SchS, in addition to anti-IL-1 therapies and bruton tyrosine kinase (BTK) inhibitors.<\/jats:p>","DOI":"10.3389\/fimmu.2025.1520470","type":"journal-article","created":{"date-parts":[[2025,1,28]],"date-time":"2025-01-28T06:42:01Z","timestamp":1738046521000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":5,"title":["Case report: Therapeutic use of bortezomib in a patient with Schnitzler 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