{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T23:22:34Z","timestamp":1788304954933,"version":"build-2803163510"},"reference-count":21,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2017,8,2]],"date-time":"2017-08-02T00:00:00Z","timestamp":1501632000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Eur J Clin Investigation"],"published-print":{"date-parts":[[2017,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Familial Mediterranean Fever (<jats:styled-content style=\"fixed-case\">FMF<\/jats:styled-content>) is an autosomal recessive autoinflammatory disorder characterised by recurrent attacks of fever and serositis (peritonitis, pleuritic or synovitis) affecting mainly populations of Mediterranean origin.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aim<\/jats:title><jats:p>To describe a relatively new cluster of <jats:styled-content style=\"fixed-case\">FMF<\/jats:styled-content> subjects from Apulia and Basilicata regions (southern Italy).<\/jats:p><\/jats:sec><jats:sec><jats:title>Patients and methods<\/jats:title><jats:p>Subjects were screened for <jats:styled-content style=\"fixed-case\">FMF<\/jats:styled-content> using the Tel\u2010Hashomer criteria and genetic analysis. Demographic data were taken from patients\u2019 files and direct interviews. Patients were investigated about attack duration, intensity and site, body temperature, skin manifestations and overall quality of life before and after treatment with colchicine. Inflammatory parameters were also measured between these periods.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Forty\u2010nine subjects had <jats:styled-content style=\"fixed-case\">FMF<\/jats:styled-content> (M : F = 26 : 23, age 38 years \u00b1 2 <jats:styled-content style=\"fixed-case\">SE<\/jats:styled-content>) and followed\u2010up up to 8 years. The age at disease onset was 22\u00b71 years \u00b1 1\u00b72<jats:styled-content style=\"fixed-case\">SE<\/jats:styled-content> and the diagnostic delay was 15\u00b75 years \u00b1 1\u00b79<jats:styled-content style=\"fixed-case\">SE<\/jats:styled-content>. The majority of patients (82%) suffered from abdominal pain, and 35% had undergone prior abdominal surgery or laparotomy. Severity score (<jats:styled-content style=\"fixed-case\">ISSF<\/jats:styled-content>) was mild in 43% of patients and intermediate in 57% of patients. Serum amyloid A (SAA) was increased in 20% of patients (16\u00b79 \u00b1 3\u00b77, normal range &lt; 6\u00b74 mg\/dL). In over 95% of patients, inflammation markers, duration and intensity of febrile painful attacks, quality of life and <jats:styled-content style=\"fixed-case\">ISSF<\/jats:styled-content> score improved dramatically following colchicine treatment.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The Apulia region represents a new endemic area for <jats:styled-content style=\"fixed-case\">FMF<\/jats:styled-content>. Clinical presentation of <jats:styled-content style=\"fixed-case\">FMF<\/jats:styled-content> can be misleading and requires a complete and early workup to recognise the disease and avoid unjustified surgery. Colchicine remains the gold standard therapy to prevent <jats:styled-content style=\"fixed-case\">FMF<\/jats:styled-content> attacks and fatal long\u2010term complications.<\/jats:p><\/jats:sec>","DOI":"10.1111\/eci.12783","type":"journal-article","created":{"date-parts":[[2017,7,5]],"date-time":"2017-07-05T15:43:19Z","timestamp":1499269399000},"page":"622-629","source":"Crossref","is-referenced-by-count":23,"title":["A novel cluster of patients with Familial Mediterranean Fever (FMF) in southern Italy"],"prefix":"10.1111","volume":"47","author":[{"given":"Leonilde","family":"Bonfrate","sequence":"first","affiliation":[{"name":"Department of Biomedical Sciences &amp; Human Oncology University \u201cAldo Moro\u201d of Bari Medical School Bari Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuseppe","family":"Scaccianoce","sequence":"additional","affiliation":[{"name":"Gastrointestinal Endoscopy \u201cUmberto I\u201d Hospital Altamura Bari Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuseppe","family":"Palasciano","sequence":"additional","affiliation":[{"name":"Department of Biomedical Sciences &amp; Human Oncology University \u201cAldo Moro\u201d of Bari Medical School Bari Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eldad","family":"Ben\u2010Chetrit","sequence":"additional","affiliation":[{"name":"FMF Clinic Hadassah\u2010Hebrew University Medical Center Jerusalem Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5359-1471","authenticated-orcid":false,"given":"Piero","family":"Portincasa","sequence":"additional","affiliation":[{"name":"Department of Biomedical Sciences &amp; Human Oncology University \u201cAldo Moro\u201d of Bari Medical School Bari Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,8,2]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng0997-25"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(00)80539-5"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/eci.12170"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.2174\/0929867323666151117121706"},{"key":"e_1_2_7_6_1","unstructured":"Infevers.Infevers: an online database for autoinflammatory mutations. 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