Demographic, clinical and mutational characteristics of Turkish familial Mediterranean fever patients: results of a single center in Central Anatolia

Clinical and genetic findings of familial Mediterranean fever (FMF) may be variable in different populations. Environmental factors may also affect phenotypic features of FMF. In this study, we investigated demographic, clinical and mutational features of FMF patients who were treated in a single re...

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Veröffentlicht in:Rheumatology international 2010-05, Vol.30 (7), p.911-915
Hauptverfasser: Üreten, Kemal, Gönülalan, Gülsüm, Akbal, Erdem, Güneş, Fahri, Akyürek, Ömer, Özbek, Mustafa, Öztürk, M. Akif
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Sprache:eng
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Zusammenfassung:Clinical and genetic findings of familial Mediterranean fever (FMF) may be variable in different populations. Environmental factors may also affect phenotypic features of FMF. In this study, we investigated demographic, clinical and mutational features of FMF patients who were treated in a single reference hospital in Turkey. Two hundred and sixty patients (169 females, 91 males, mean age 30.44 ± 10.29 years) were included in this study. All patients were evaluated regarding MEFV gene mutations. The mean age of disease onset was 17.21 ± 8.66 years (range 2–40 years). The mean duration between the disease onset and diagnosis was 9.39 ± 8.92 years. Seventy percent of patients had symptoms before 20 years of age (early onset FMF). Arthritis and erysipelas like erythema (ELE) were more common, and the mean duration between the disease onset and diagnosis was longer in early onset FMF patients. The frequency of attacks per year, and disease severity score (DSS) was higher in early onset patients. Homozygote mutation of M694V was detected in 37 (20.2%) and 4 (5.2%) patients in early onset FMF and adult onset FMF groups, respectively ( p  
ISSN:0172-8172
1437-160X
DOI:10.1007/s00296-009-1073-6