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OBJECTIVE: The aim of this study was to perform a systematic review of the relevant literature aiming to assess the role of MEFV mutations on FMF-associated PAN.
MATERIAL and METHODS: We conducted a comprehensive review of the literature with an attempt to analyze cumulated data regarding the role of MEFV mutations in the development of FMFassociated PAN.
RESULTS: We found a total of 96 cases with FMF and PAN. MEFV mutations were available only in 28 patients of whom 26 have been reported from Turkey. Twenty-five (89 %) of the 28 patients had at least one M694V allele and 13 (46%) of them had the homozygous M694V genotype.
CONCLUSION: Since M694V is accepted to be associated with more severe inflammation as compared to other mutations, one can speculate that this enhanced inflammation may predispose to PAN and MEFV mutations and probably contribute to the risk of developing PAN in areas where FMF is endemic. In addition, MEFV mutations, particularly M694V, might be searched in patients from certain ethnic groups, especially in young patients having PAN without any predisposing disease.