Acta paediatrica (Oslo, Norway : 1992) | 1 Dec 2012
P Wekell, V Friman, B Balci-Peynircioglu, E Yilmaz, A Fasth and S Berg
AIM: To characterize Familial Mediterranean Fever (FMF) in western Sweden, focusing on, genotype, clinical picture, prevalence and age of onset as well as time to diagnosis. METHODS: Patients with autoinflammatory diseases are continuously registered at the five main hospitals in Western Sweden. Case records of patients with FMF were analysed retrospectively. Population data on immigration was retrieved from Statistics Sweden. RESULTS: Until 2008, 37 patients with FMF were identified. The prevalence among inhabitants of Turkish, Lebanese, Syrian and Iranian origin was 173, 124, 86 and 17/100 000, respectively. Median age at first symptoms was 4 years (range 3 month - 37 years) and at diagnosis 10 years (range 2 - 44 years). Median time from first symptoms to diagnosis was 4 years (range < 1 year to 34 years). Among 32 patients screened for twelve common mutations, 75% were homozygotes or compound heterozygotes, 16% were heterozygotes and in 9% no mutation was found. In our cohort the frequencies of symptoms were fever 100%, peritonitis 90%, pleuritis 22% and arthritis 12%. CONCLUSIONS: The majority of patients with FMF present during childhood. The prevalence among immigrants in western Sweden is in the same range as in their country of origin. Time to diagnosis needs to be shortened by means of increased awareness of the disease. ©2012 The Author(s)/Acta Paediatrica ©2012 Foundation Acta Paediatrica.
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