Amyloidosis and its related factors in Turkish patients with familial Mediterranean fever: a multicentre study

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dc.authorid0000-0001-7737-4180en_US
dc.authorid0000-0002-0783-1072en_US
dc.authorid0000-0002-9035-689Xen_US
dc.contributor.authorKaşifoğlu, Timuçin
dc.contributor.authorBilge, Şule Yaşar
dc.contributor.authorSarı, İsmail
dc.contributor.authorSolmaz, Dilek
dc.contributor.authorŞenel, Soner
dc.contributor.authorBes, Cemal
dc.date.accessioned2021-06-23T19:36:09Z
dc.date.available2021-06-23T19:36:09Z
dc.date.issued2014
dc.departmentBAİBÜ, Tıp Fakültesi, Dahili Tıp Bilimleri Bölümüen_US
dc.description.abstractMethods. Fifteen centres from the different geographical regions of Turkey were included in the study. Detailed demographic and medical data based on a structured questionnaire and medical records were collected. The diagnosis of amyloidosis was based on histological proof of congophilic fibrillar deposits in tissue biopsy specimens. Results. There were 2246 FMF patients. The male/female ratio was 0.87 (1049/1197). The mean age of the patients was 34.5 years (s.d. 11.9). Peritonitis was the most frequent clinical finding and it was present in 94.6% of patients. Genetic testing was available in 1719 patients (76.5%). The most frequently observed genotype was homozygous M694V mutation, which was present in 413 (24%) patients. Amyloidosis was present in 193 patients (8.6%). Male sex, arthritis, delay in diagnosis, M694V genotype, patients with end-stage renal disease (ESRD) and family history of amyloidosis and ESRD were significantly more prevalent in patients with amyloidosis compared with the amyloidosis-negative subjects. Patients with homozygous M694V mutations had a 6-fold higher risk of amyloidosis compared with the other genotypes (95% CI 4.29, 8.7, P < 0.001). Conclusion. In this nationwide study we found that 8.6% of our FMF patients had amyloidosis and homozygosity for M694V was the most common mutation in these patients. The latter finding confirms the association of homozygous M694V mutation with amyloidosis in Turkish FMF patients.en_US
dc.identifier.doi10.1093/rheumatology/ket400
dc.identifier.endpage745en_US
dc.identifier.issn1462-0324
dc.identifier.issn1462-0332
dc.identifier.issue4en_US
dc.identifier.pmid24369413en_US
dc.identifier.scopus2-s2.0-84897830773en_US
dc.identifier.scopusqualityQ1en_US
dc.identifier.startpage741en_US
dc.identifier.urihttps://doi.org/10.1093/rheumatology/ket400
dc.identifier.urihttps://hdl.handle.net/20.500.12491/7941
dc.identifier.volume53en_US
dc.identifier.wosWOS:000333263500025en_US
dc.identifier.wosqualityQ1en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.institutionauthorBes, Cemal
dc.language.isoenen_US
dc.publisherOxford Univ Pressen_US
dc.relation.ispartofRheumatologyen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.subjectFamilial Mediterranean Feveren_US
dc.subjectAmyloidosisen_US
dc.subjectPrevalenceen_US
dc.titleAmyloidosis and its related factors in Turkish patients with familial Mediterranean fever: a multicentre studyen_US
dc.typeArticleen_US

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