POS1359 REGIONAL DIFFERENCES IN DISEASE CHARACTERISTICS OF FAMILIAL MEDITERRANEAN FEVER IN TURKEY: PRELIMINARY REPORT

Background: Familial Mediterranean fever (FMF) is a hereditary autoinflammatory disease accompanied by recurrent attacks of and serositis. It prevalent among populations, mainly Turks, Armenians; Jews Arabs. As genetic factors are variable in the population, environmental can also affect phenotypic characteristics. Objectives: This study aimed to determine geographic differences characteristics burden patients with FMF Turkey. Methods: Patients diagnosed according Tel-Hashomer criteria were included this multi-center study. from different regions Demographic features clinic including duration, medications, comorbid conditions, attack characteristics, amyloidosis, acute phase reactants, gene mutations, arthritis, sacroiliitis, febrile myalgia recorded. PRASS activity score, FMF-QoL, HAD, HAQ assessed. parts Turkey divided into 3 groups Central Anatolia, Western, Eastern. Disease activity, investigated distinct regions. Results: A total 281 (195 women, 86 men) enrolled The mean age was 34.9 (SD:12.3) years. While eastern areas earlier ( p<0.001 ), western area had longer diagnostic delay time ). tended have higher ESR scores than those central Anatolian regions, but numbers per 6 months similar highest proportion who M694V/M694V homozygous western, then Anatolia (19.5%, 18%, 5.4%). arthritis more common eastern, pleuritis sacroiliitis anatolia. Peritonitis erysipelas like erythema rates majority receiving colchicine treatment all three FMF-QoL lowest p=0.006 region experienced functional disability (p=0.009). Anxiety depression between (p=0.385 vs p=0.549). Conclusion: These findings suggest that diversity concerning at diagnosis, time, quality life, In genotypic analysis, M694V mutation most pathogenic Regional varieties may explain areal differences. relationships occur clearly larger patient populations. References: [1]Ureten K,et al. Demographic, clinical mutational Turkish familial patients: results single center Anatolia. Rheumatol Int . 2010;30(7):911-915. Table 1. Clinical demographic n=107 Central, n=74 Eastern, n=100 p Age 37.7 (12.5) 36 (12.6) 31.2 (10.9) < 0.001 Sex, female 79 (73.8%) 58 (78.4%) (%58) 0.007 symptom onset, year 16.8 (11.3) 20.4 (13.2) 18.5 (11) 0.229 29.6 (13.4) 27.7 (13.3) 22.1 (12.1) Diagnostic 12.3 (12.9) 7.3 (10.5) 3.6 (5.3) ESR, mm/hr 26.8 (18.9) 15.5 (12) 13.1 (13.1) 0.003 41 (55.4%) 0.577 Low 50 (46.7%) 28 (37.8%) 51 (51%) Moderate 48 (44.9%) 45 (45%) High 9 (8.4%) 5 (6.8%) 4 (4%) Fever 83 (77.6%) 60 (81.1%) 96 (96%) 98 (91.6%) 64 (86.55) 92 (92%) 0.412 Pleuritis 35 (32.7%) 27 (36.5%) 18 (18%) 0.013 Arthritis (16.8) 16 (21.6) (36%) 0.005 Sacroiliitis 8 (7.5%) 14 (18.9%) 1 (1%) 0.000 Erysipelas 2 (1.9%) 7 (9.5%) (7%) 0.075 Data presented as (SD), median (min-max), n (% ) Disclosure Interests: None declared