Date: Monday, October 22, 2018
Session Type: ACR Poster Session B
Session Time: 9:00AM-11:00AM
Familial Mediterranean Fever (FMF) is the most frequent auto-inflammatory disease caused by MEFV gene mutations. Although FMF is characterized by intermittent inflammatory attacks some patients exert chronic persistent inflammation that can result in damage of multiple organs. Available reports investigated only specific components of damage such as amyloidosis. All possible organ targets of damage have not been entirely evaluated before.
To investigate all components of FMF related damage and associated factors with damage.
Methods: All patients recruited from FMF in Central Anatolia (FiCA) cohort, currently comprising 664 adult subjects (mean age 36±12.1, 63.5% female). All patients fulfilled Tel Hashomer criteria and all were using colchicine for at least 1 year. Demographic data, FMF disease and mutation (if available) characteristics and treatment features were recorded. FMF related damage was evaluated with recently validated auto-inflammatory disease damage index (ADDI). Association between damage and demographic, disease and treatment characteristics were analyzed.
Results: Proportions of FMF manifestations were fever 81,2%, peritonitis 90%, pleuritis 48.8%, arthritis 54.4% and skin rash 29%. Dominant attack types were fever in 7.1%, serositis in 62.7%, musculoskeletal in 16.7% and all attacks were common in rest of patients. MEFV mutations were available in 536 subjects and 77.6% of subjects harboring M694V mutation (28.8% homozygous for M694V). Among all 59.5% patients were well responded to colchicine and 9.2% 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 52.4%, joint deformity in 2.7%, infertility in 11.2%, amenorrhea in 7.1%, proteinuria in 7.8%, amyloidosis in 6.8% and severe renal failure in 4.1%. Age, M694V homozygous mutation, presence of persistent inflammation, dominant musculoskeletal attacks and colchicine nonresponse were found to be the independent predictors of damage.
Conclusion: M694V homozygous mutation, persistent inflammation, colchicine non-response and musculoskeletal dominant attacks are predictors of damage and effective therapeutic interventions must be undertaken to prevent from damage in these patients.
To cite this abstract in AMA style:Tufan A, Armagan B, Bodakci E, Kasifoglu T, Satis H, Atas N, Sari A, Babaoglu H, Yardımcı G, Salman R, Kilic L, Yasar Bilge NS, Ozturk MA. Familial Mediterranean Fever Related Damage Assessed By Auto-Inflammatory Disease Damage Index (ADDI) and Associated Factors with Damage [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 10). https://acrabstracts.org/abstract/familial-mediterranean-fever-related-damage-assessed-by-auto-inflammatory-disease-damage-index-addi-and-associated-factors-with-damage/. Accessed January 22, 2020.
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