FRI0617 APPLICATION OF AUTO-INFLAMMATORY DISEASE DAMAGE INDEX (ADDI) TO PATIENTS WITH FMF AND RELATED FACTORS WITH DAMAGE

Abstract

Background Familial Mediterranean Fever (FMF) is the most frequent auto-inflammatory disease caused by MEFV gene mutations. Available reports investigated only specific components of damage such as amyloidosis. All possible organ targets of damage have not been entirely evaluated before. Such as Disease severity index which is emerged especially for FMF do not cover entire damage domains related to FMF. Recently, a new scoring system (Auto-inflammatory disease damage index) was developed and validated for autoinflammatory diseases. Objectives We aimed to investigate damage accrual caused by FMF and associated features with damage. Methods All patients recruited from FMF in Central Anatolia (FiCA) cohort, which is a duplication disabled, internal and externally controlled, cross-sectional, multicenter accessible web-based cohort. This study is comprising 970 adult patients (mean age 35.3 ±12.1 years, 61.5% female). Demographic data, FMF disease characteristics, co-morbid conditions, disease complications were meticulously questioned and laboratory features and genotype data (if available) were recruited from patient files. FMF caused damage was assessed by auto-inflammatory disease damage index (ADDI) which is recently validated. Association between damage and demographic, disease and treatment characteristics were analyzed. Results Proportions of FMF manifestations were fever 83.1%, peritonitis 91.5%, pleuritis 47.9%, arthritis 43.3% and skin rash 26.2%. Dominant attack types were fever in 6.2%, serositis in 65.7%, musculoskeletal in 16.8% and all types of attacks were common in rest of patients. MEFV mutations were available in 814 subjects and 75.9% of these subjects were harboring M694V mutation (42.5% homozygous for M694V). Among all 63.1% patients were well responded to colchicine and 8.8% were non-responders. Median ADDI score was 1 (min 0-max 11). Most common FMF related damages were observed in musculoskeletal, reproductive and kidney domains. Chronic musculoskeletal pain was present in 49%, joint deformity in 2.9%, infertility in 6.6%, amenorrhea in 3.9%, proteinuria in 6.9%, amyloidosis in 5.9% and renal failure in 3.7% of the patients. 411 (%42.3) of patients had no damage accrual. M694V homozygous mutation, male gender and colchicine nonresponse were found to be the independent predictors of damage. Conclusion M694V homozygous mutation, colchicine non-response and male gender are predictors of damage and effective therapeutic interventions must be undertaken to prevent from damage in these patients. Acknowledgement None Disclosure of Interests Hakan Babaoglu: None declared, Berkan Armagan: None declared, Erdal Bodakci: None declared, Timucin Kasifoglu: None declared, Hasan Satis: None declared, Nuh Atas: None declared, Alper Sari: None declared, Nazife Sule Yasar Bilge: None declared, Gozde Kubra Yardimci: None declared, reyhan bilici salman: None declared, Levent Kilic: None declared, mehmet akif ozturk: None declared, Berna Goker: None declared, seminur haznedaroglu: None declared, Umut Kalyoncu Grant/research support from: MSD, Roche, UCB, Novartis and Pfizer, Consultant for: MSD, Abbvie, Roche, UCB, Novartis, Pfizer and Abdi Ibrahim, Speakers bureau: MSD, Abbvie, Roche, UCB, Novartis, Pfizer and Abdi Ibrahim, abdurrahman tufan: None declared