Frequency of rheumatic diseases in patients with familial Mediterranean fever

Purpose: Mutations in the Mediterranean FeVer (MEFV) gene, which causes familial fever (FMF), may also cause emergence of other specific rheumatic diseases. This study aims to determine frequency rheumatologic diseases paediatric FMF patients, evaluate whether there are clinical and genetic differences between those with without concomitant diseases, compare data previous studies.
 Materials methods: The files patients who were followed up at rheumatology department reviewed retrospectively. Demographic data, MEFV mutations, treatment, disease severity scores, recorded from files.
 Results: There 303 (154 female/149 male). mean age diagnosis was 7.04±3.9 years. duration 5.33±3.13 In cohort, 41 (13.5%) diagnosed another disease. 22 cases juvenile idiopathic arthritis (53.6%), seven vasculitis (17%), six periodic aphthous stomatitis adenitis syndrome (14.6%), three Behçet (7.3%), two acute (4.8%), one case systemic lupus erythematosus (2.4%). 32 these (78%) had M694V mutation (homozygous 11, heterozygous 21). Disease activity scores PRAS ISSF higher (p=0.002 p