Background:Familial Mediterranean Fever (FMF), which is more common in groups in the Mediterranean basin, is a monogenic auto inflammatory disease characterized by recurrent attacks of febrile peritonitis, pleuritis and arthritis.Objectives:The aim of this study is to investigate the clinical features of patients diagnosed with juvenile and adult-onset Familial Mediterranean Fever (FMF).Methods:Patients with FMF were included in the study consecutively without sample selection. Data about age, sex, disease duration (month), symptom duration, age at diagnosis, diagnosis delay time, comorbid diseases, and medications were noted. Patients with onset of symptoms ≤ 20 years old were classified as juvenile-onset, those > 20 years old were classified as adult-onset FMF. The frequency and characteristics of attacks and the presence of amyloidosis will be recorded.Disease activity was assessed with the PRAS disease activity score. The Health Assessment Questionnaire (HAQ) and SF-36 were used to evaluate physical disability and quality of life, respectively.The descriptive analysis was done for all parameters. Differences between categorical variables were assessed by Chi-square test and Fisher’s exact test. The Mann Whitney-U test was used to compare two sample means. P<0.05 accepted as significant. SPSS 20.0 (Statistical package for social sciences for Windows 20.0) program was used for the statistical analysis.Results:The mean age of 86 patients (63 female, 23 male) with FMF was 38.38 (SD: 12.13) years. The patients with the juvenile-onset FMF were 26.7% of the patients.There were no differences between juvenile and adult-onset FMF groups in terms of gender, frequency of attacks, duration of attacks, acute phase values between attacks, colchicine dose, presence of colchicine resistance, and presence of amyloidosis (p>0.05). The latency to diagnosis was significantly higher in patients with adult-onset FMF (p<0.005).The PRAS disease activity scores were significantly higher in the juvenile-onset FMF group (p=0.001). There were no significant differences between the two groups in terms of SF-36 and HAQ scores (p> 0.05).Conclusion:While there were no differences between juvenile and adult-onset FMF patients in terms of quality of life and functional disability, the PRAS disease activity scores were higher in patients with juvenile-onset FMF.References:[1]Shinawi M, Brik R, Kepten I, Berant M, Gersoni B. Familial Mediterranean fever: high gene frequency and heterogenous disease among Israeli-Moslem Arab population. J Rheumatol 2000;27:1492–5.Disclosure of Interests:None declared
AB1024 EVALUATION OF CLINICAL FEATURES IN PATIENTS DIAGNOSED WITH JUVENILE AND ADULT-ONSET FAMILIAL MEDITERRANEAN FEVER
