AB0993 COMORBIDITIES IN FAMILIAL MEDITERRANEAN FEVER
Authors: Ummusen Kaya Akca, Banu Balci Peynircioglu, Zehra Serap Arici, Edibe Avci, Zulfiye Yeliz Akkaya Ulum, Engin Yilmaz, Yelda Bilginer, Seza özen
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Familial Mediterranean Fever (FMF) is a periodic fever syndrome, characterized by recurrent episodes of fever and serosal inflammation accompanied with high acute phase reactants.The analysis of possible comorbidities is important to understand the impact of these conditions on clinical care and whether they share a common etiological pathway. <h3>Objectives</h3> We aimed to evaluate the comorbidities associated with FMF patients in a large genetically diagnosed cohort. <h3>Methods</h3> We retrospectively evaluated the medical records of FMF patients who were followed up at Department of Pediatric Rheumatology in Hacettepe University between 2000 and 2015. This study was approved by the Research Ethics Committee and was conducted in accordance with the Declaration of Helsinki. The diagnosis of FMF was made according to Tel Hashomer diagnosis criteria for patients who applied prior to April 2009 and to the Turkish FMF pediatric diagnosis criteria after April 2009. The FMF patients who had homozygous or compound heterozygous mutations were included in the study. Comorbidities associated with FMF were divided into three groups; associated with increased inflammation, associated with FMF and incidental. <h3>Results</h3> A total of 1999 patients were enrolled in the study. Of all 1999 FMF patients, 636 were children (31.8%), 1029 were males (51.4%), with a mean age of 31.60±16.01 years. The mean follow up time was 4.50±3.99 years (median:3.84 range from 0.21-29.4 years). 880 of 1999 (44%) FMF patients had homozygous MEFV gene mutation, the most common mutation was M694V homozygous. The remaining were compound heterozygous. 656 patients (32.8%) had one or more than one comorbidity associated with FMF. Ankylosing spondylitis was the most common comorbidity associated with increased inflammation while the most common comorbidity in FMF related comorbidities was renal amyloidosis. The frequency of ankylosing spondylitis, henoch schonlein purpura, juvenile idiopathic arthritis, polyarteritis nodosa (PAN), multiple sclerosis (MS) and Behçet's disease were increased in patients with FMF when compared to those in the literature. Systemic lupus erythematosus was observed less frequently in the patients with FMF than in the population. While the increase in the frequency of MS was 3.3 times, the frequency of PAN was increased 110 times. <h3>Conclusion</h3> This study shows that FMF is a hereditary disease associated with significant comorbidity. We also confirm that inflamatory and rheumatic diseases are more common in FMF. <h3>Disclosure of Interests</h3> Ummusen Kaya Akca: None declared, Banu Balci Peynircioglu: None declared, Zehra Serap Arici: None declared, Edibe Avci: None declared, Zulfiye Yeliz Akkaya Ulum: None declared, Engin Yilmaz: None declared, Yelda Bilginer: None declared, Seza özen Consultant for: Seza Ozen is receiving consultancy fees from Novartis, Speakers bureau: Roche