THU0562 PREVALENCE, UNDERLYING FACTORS AND DAMAGE ASSOCIATED WITH CHRONIC PERSISTENT INFLAMMATION IN PATIENTS WITH FMF
Authors: Hakan Babaoglu, Berkan Armagan, Erdal Bodakci, Timuçin Kaşifoğlu, Hasan Satış, Nuh Atas, Alper Sarı, Nazife Sule Yasar Bilge, Gözde Kübra Yardımcı, Reyhan Bilici Salman, Levent Kılıç, Mehmet Akif Ozturk, Berna Goker, Seminur Haznedaroglu, Umut Kalyoncu, abdurrahman tufan
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background:</h3> Familial Mediterranean Fever (FMF) is the most frequent auto-inflammatory disease characterized by recurrent, self-limiting attacks of fever, peritonitis, pleuritis, synovitis, myalgia, and erysipelas-like erythema (ELE). Attacks has a robust inflammatory response which completely normalize when attacks subside. However, substantial number of FMF patients possess chronic persistent inflammation even in between attacks. Clinical significance of persistent inflammation and its contribution to damage accrual are yet to be determined. <h3>Objectives:</h3> We aimed to determine the prevalence and underlying factors of chronic persistent inflammation and its association with the domains of Auto-inflammatory disease damage index (ADDI) in a large cross-sectional multicenter cohort. <h3>Methods:</h3> 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. Demographic data, disease characteristic, attack features (ever presented) were meticulously questioned. Genotype data (if available) and laboratory features including inflammatory markers were recorded. FMF related damage scores was assessed by auto-inflammatory disease damage index (ADDI) which is recently validated. Patients were stratified according to their antecedent acute phase responses as having persistent inflammation (PI group) or not, both groups compared in terms of disease characteristics, attack features, inflammatory comorbidities and damage domains. <h3>Results:</h3> Study is comprised 970 adult patients (mean age 35.3±12.1, 61% female). 54 of them were excluded for their first inclusion. 15% of patients had persistent inflammation. PI group had significantly younger age of diagnosis, male dominance, homozygous M694V, more frequent attacks it the last year, more pleuritis, arthritis, myalgia and ELE and more severe disease according to ISSF than other patients (Table-1). Moreover, colchicine resistance and ADDI damage scores were remarkably higher in PI group. In multi-variate analyzes colchicine resistance OR=2.71 [95%CI 1.38-5.35], ISSF OR=11.06 [95%CI 5.23-23.39], male gender OR= 2.38 [95%CI 1.51-3.76], homozygous M694V mutation OR= 2.31 [95%CI 1.41-3.78] and ELE OR= 1.72 [95%CI 1.04-2.857] found as independent predictors of persistent inflammation. PI group had more damage in multiple domains like joint restriction, musculoskeletal pain, proteinuria, amyloidosis, renal insufficiency and developmental delay. Same variables and persistent inflammation further analyzed in in terms of predicting the damage. Persistent inflammation found to be an independent predictor of proteinuria OR=2.02 [95%CI 1.05-3.90], amyloidosis OR=2.71 [95%CI, 1.34-5.48]), and renal insufficiency OR=4.36 [95%CI 1.84-10.32]. <h3>Conclusion:</h3> Persistent inflammation is relatively common in FMF patients particularly in those harboring M694V homozygous mutation and is associated with major complications of disease indicating poor prognosis. <h3>Acknowledgement:</h3> None <h3>Disclosure of Interests:</h3> Hakan Babaoglu: None declared, Berkan Armagan: None declared, Erdal Bodakci: None declared, Timuçin Kaşifoğlu: None declared, Hasan Satış: None declared, Nuh Atas: None declared, Alper Sarı: None declared, Nazife Sule Yasar Bilge: None declared, Gözde Kübra Yardımcı: None declared, reyhan bilici salman: None declared, Levent Kılıç: 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