{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,6]],"date-time":"2026-07-06T10:14:44Z","timestamp":1783332884567,"version":"3.54.6"},"reference-count":38,"publisher":"Oxford University Press (OUP)","issue":"4","license":[{"start":{"date-parts":[[2025,12,24]],"date-time":"2025-12-24T00:00:00Z","timestamp":1766534400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,6,30]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>Familial Mediterranean Fever (FMF) is characterized by recurrent fever and serositis. Although not classical in FMF, vasculitis is increasingly reported, suggesting shared pathogenic mechanisms. This study aimed to evaluate clinical features and treatment responses in paediatric FMF patients with concomitant vasculitis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>The records of 1598 paediatric FMF patients followed at a tertiary paediatric rheumatology centre were reviewed retrospectively. After exclusions, 1481 patients were included: 1434 without vasculitis (Group 1) and 47 with vasculitis (Group 2). Demographic, clinical, genetic, and treatment-related data were compared between the groups, and vasculitis subtypes were characterized in detail.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Vasculitis was found in 3.2% of patients, most commonly IgA vasculitis and Beh\u00e7et\u2019s disease. Group 2 had higher attack rates, more chest pain\/myalgia, and more non-exon 10 heterozygous Mutations in the Mediterranean Fever mutations. Colchicine response and biologic therapy requirement did not differ significantly between groups. There was no statistically significant difference between groups in colchicine response (97.3 versus 74.5%) or biologic therapy requirement (2.7 versus 6.4%).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>The coexistence of vasculitis in FMF likely reflects shared inflammatory mechanisms rather than coincidence. Vasculitis occurred even in patients with milder Mutations in the Mediterranean Fever genotypes, suggesting an enhanced proinflammatory milieu. Early recognition of vasculitis in FMF may influence therapeutic strategies and improve outcomes.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/mr\/roaf128","type":"journal-article","created":{"date-parts":[[2025,12,23]],"date-time":"2025-12-23T12:51:16Z","timestamp":1766494276000},"page":"648-656","source":"Crossref","is-referenced-by-count":0,"title":["Clinical implications of Vasculitis associated with familial Mediterranean fever: a comparative study in childhood"],"prefix":"10.1093","volume":"36","author":[{"given":"B\u00fc\u015fra Ba\u015fer","family":"Ta\u015fk\u0131n","sequence":"first","affiliation":[{"name":"Division of Pediatric Rheumatology, Department of Pediatrics, Istanbul University Faculty of Medicine , Istanbul ,","place":["T\u00fcrkiye"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ay\u015fenur Do\u011fru","family":"K\u0131l\u0131n\u00e7","sequence":"additional","affiliation":[{"name":"Division of Pediatric Rheumatology, Department of Pediatrics, Istanbul University Faculty of Medicine , Istanbul ,","place":["T\u00fcrkiye"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Selen Duygu","family":"Ar\u0131k","sequence":"additional","affiliation":[{"name":"Division of Pediatric Rheumatology, Department of Pediatrics, Istanbul University Faculty of Medicine , Istanbul ,","place":["T\u00fcrkiye"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7216-0562","authenticated-orcid":false,"given":"\u00d6zlem","family":"Akg\u00fcn","sequence":"additional","affiliation":[{"name":"Division of Pediatric Rheumatology, Department of Pediatrics, Istanbul University Faculty of Medicine , Istanbul ,","place":["T\u00fcrkiye"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3594-7387","authenticated-orcid":false,"given":"Nuray Aktay","family":"Ayaz","sequence":"additional","affiliation":[{"name":"Division of Pediatric Rheumatology, Department of Pediatrics, Istanbul University Faculty of Medicine , Istanbul ,","place":["T\u00fcrkiye"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2025,12,24]]},"reference":[{"key":"2026070605222595300_ref1","doi-asserted-by":"crossref","first-page":"763","DOI":"10.3389\/fimmu.2019.00763","article-title":"Association of Vasculitis and familial Mediterranean Fever","volume":"10","author":"Abbara","year":"2019","journal-title":"Front Immunol"},{"key":"2026070605222595300_ref2","doi-asserted-by":"publisher","first-page":"42","DOI":"10.1002\/art.1780250107","article-title":"Sch\u00f6nlein-Henoch syndrome in patients with familial Mediterranean fever","volume":"25","author":"Flatau","year":"1982","journal-title":"Arthritis Rheum"},{"key":"2026070605222595300_ref3","first-page":"323","article-title":"Vasculitis in familial Mediterranean fever","volume":"24","author":"Ozdogan","year":"1997","journal-title":"J Rheumatol"},{"key":"2026070605222595300_ref4","doi-asserted-by":"publisher","first-page":"475","DOI":"10.1093\/ndt\/14.2.475","article-title":"Familial Mediterranean fever--renal involvement by diseases other than amyloid","volume":"14","author":"Tekin","year":"1999","journal-title":"Nephrol Dial Transplant"},{"key":"2026070605222595300_ref5","doi-asserted-by":"publisher","first-page":"286","DOI":"10.1016\/S0049-0172(00)80015-3","article-title":"Beh\u00e7et's disease in familial Mediterranean fever: characterization of the association between the two diseases","volume":"29","author":"Schwartz","year":"2000","journal-title":"Semin Arthritis Rheum"},{"key":"2026070605222595300_ref6","doi-asserted-by":"crossref","first-page":"1372","DOI":"10.1093\/rheumatology\/kez410","article-title":"Comorbidities in familial Mediterranean fever: analysis of 2000 genetically confirmed patients","volume":"59","author":"Balc\u0131-Peynircio\u011flu","year":"2020","journal-title":"Rheumatology (Oxford)"},{"key":"2026070605222595300_ref7","doi-asserted-by":"crossref","first-page":"395","DOI":"10.1093\/rheumatology\/ken509","article-title":"A new set of criteria for the diagnosis of familial Mediterranean fever in childhood","volume":"48","author":"Yal\u00e7inkaya","year":"2009","journal-title":"Rheumatology (Oxford)"},{"key":"2026070605222595300_ref8","doi-asserted-by":"crossref","first-page":"1025","DOI":"10.1136\/annrheumdis-2019-215048","article-title":"Classification criteria for autoinflammatory recurrent fevers","volume":"78","author":"Gattorno","year":"2019","journal-title":"Ann Rheum Dis"},{"key":"2026070605222595300_ref9","doi-asserted-by":"crossref","first-page":"790","DOI":"10.1136\/ard.2009.116624","article-title":"EULAR\/PRINTO\/PRES criteria for Henoch-Sch\u00f6nlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. 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