SAT0523 EVALUATION OF COEXISTING DISEASES IN CHILDREN WITH FAMILIAL MEDITERRANEAN FEVER
Authors: Mehmet Yildiz, Amra Adrovic, Emre Tasdemir, Khanim Baba-Zada, Muhammed Aydin, Oya Koker, Sezgin Sahin, Kenan Barut, Ozgur Kasapcopur
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Familial Mediterranean Fever (FMF) is most common periodic fever syndrome in childhood. It is characterized by fever attacks, abdominal pain lasting between 6 - 72 hours, serositis and erysipelas like erythema. Since FMF is inherited in autosomal recessive manner, it has higher frequency in populations that have higher rate of consanguineous marriages. As it is a lifelong chronic disorder, it is important to understand its clinical course for preventive medicine. Despite the higher incidence of variety of disorders shown studies among adult FMF patient, there is not enough data from pediatric populations. <h3>Objectives</h3> The objective of the study is to evaluate the comorbid disorders in a large pediatric familial Mediterranean fever cohort. <h3>Methods</h3> Six hundred and eighty-six children with FMF were interviewed by the same investigator between October 2018 and January 2019 in our pediatric rheumatology department. Demographic features and MEFV gene mutations were recorded from patients' charts. Patients and/or their parents were asked about characteristics of their fever episodes, presence of arthralgia, arthritis, abdominal pain, chest pain in the course of fever attack and coexistence of any other disease confirmed by a physician. <h3>Results</h3> Female-male ratio of our cohort was 0,85. Mean age of the patients was 12,8 ± 7,4 years. Mean age of patients at disease onset and at the time of diagnosis was 4,38 ± 3,46 years and 6,55 ± 3,67 years, respectively. Sixty-five and a half percent of patients had family history of FMF. Consanguineous marriage rate was 29,8%. Most common MEFV mutations were: M694V homozygotes (18,8%), M694V heterozygotes (17,7%), R202Q heterozygotes (13,1%), R202Q homozygotes (5,5%) and V726A heterozygotes (4,9%), respectively. Resistance to colchicine treatment was present in 43 (6,2%) patients. Number of patients that underwent tonsillectomy were 9 (1,3%) and number of patients that had appendectomy was 11 (1,6%). Detected coexisting diseases are listed in <b>Table 1</b>. <h3>Conclusion</h3> In this study, we have reported increased frequency of juvenile idiopathic arthritis, asthma- reactive airway disease, Henoch Schonlein purpura, uveitis, inflammatory bowel disease, PFAPA syndrome and acute rheumatic fever in a large pediatric FMF cohort. Those findings consistent with data from literature. It is important to be alert about these diseases that may develop in patients with FMF for preventing them from the potential morbidities. <h3>References</h3> [1] Barut K, Sahin S, Adrovic A, et al. (2018). Familial Mediterranean fever in childhood: a single-center experience. Rheumatology international, 38(1), 67-74. [2] Beser ÖF, Kasapçopur Ö, Çokugras F Ç, et al. (2013). Association of inflammatory bowel disease with familial Mediterranean fever in Turkish children. Journal of pediatric gastroenterology and nutrition, 56(5), 498-502. [3] Barut K, Sahin S, Kasapcopur O. (2016). Pediatric vasculitis. Current opinion in rheumatology, 28(1), 29-38. [4] Pehlivan E, Adrovic A, Sahin S, et al. (2018). PFAPA Syndrome in a Population with Endemic Familial Mediterranean Fever. The Journal of pediatrics, 192, 253-255. <h3>Disclosure of Interests</h3> None declared