{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T16:34:31Z","timestamp":1791304471848,"version":"4.3.1"},"reference-count":29,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2025,8,12]],"date-time":"2025-08-12T00:00:00Z","timestamp":1754956800000},"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:sec><jats:title>Introduction<\/jats:title><jats:p>Various types of vasculitides have been identified in patients with familial Mediterranean fever (FMF); however, FMF characteristic in patients who experience vasculitis during the disease course have not been described. This study aimed to describe the types of vasculitides in FMF and characterize the patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This nested case-control study compared 27 patients with FMF (12 male) diagnosed with vasculitis with 100 patients (49 men) who did not develop vasculitis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Most patients (25\/27) developed vasculitis after FMF diagnosis. Four types of vasculitides were observed: cutaneous small vessel vasculitis (10 patients, 37%), Henoch\u2013Schonlein purpura\/immunoglobulin A vasculitis (8 patients, 30%), periarteritis nodosa (three patients, 11%), and Beh\u00e7et disease (six patients, 22%). The vasculitis group was younger at FMF onset (6.6 [\u00b1 5.9] years vs. 16.2 [\u00b1 13.7] years, p &amp;lt; 0.002) and diagnosis (13.1 [\u00b1 13.1] years vs. 25.1 [\u00b1 17.92] years, p &amp;lt; 0.001). This group showed a higher frequency of homozygosity for the M694V mutation (73.9% vs. 29.4%, p &amp;lt; 0.001), had a more severe FMF (mean Pras severity score: 10.4 [\u00b1 2.6] vs. 7.3 [\u00b1 3.1], p &amp;lt; 0.001), required higher colchicine doses (1.96 [\u00b1 0.61] mg\/d vs. 1.66 [\u00b1 0.65] mg\/d, p &amp;lt; 0.025), and tended to show higher rates of colchicine resistance (29.6% vs. 12%, p = 0.053). However, vasculitis was not an independent factor influencing FMF severity.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Patients with FMF and vasculitis are characterized by a more severe disease, likely due to factors other than vasculitis itself. Yet, its presence can serve as a clinical clue to disease severity.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fimmu.2025.1506457","type":"journal-article","created":{"date-parts":[[2025,8,12]],"date-time":"2025-08-12T05:31:33Z","timestamp":1754976693000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":1,"title":["Vasculitis as an indicator of disease severity in familial Mediterranean 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