Background FMF has been reported to be frequently combined with rheumatic diseases also underlane by in inflammatory process.
Objectives To describe the cases of FMF comorbid with rheumatic diseases in children of the Russian population followed up in Federal Rheumatologic Centers.
Methods All the patients with periodic fever diagnosed as FMF who had been consulted in “Nasonova Research Institute of Rheumatology” from 2008 to 2014 were included in the study. The diagnosis of FMF was verified by Livneh criteria. Comorbid rheumatic diseases were diagnosed according to the criteria generally accepted in pediatric rheumatology. As a standard in pediatric rheumatology, coding sequences of MEFV gene were analyzed by direct automatic sequencing in all the patients (investigation of “hot spots” or complete genome analysis).
Results FMF was diagnosed in 13 children, 9 girls and 4 boys. Average age of the onset was 7.0±4.4 years. Most patients (11) were the Armenians and 2 patients were related to the peoples of the Northern Caucasus. In 5 patients (38.5%) the disease combined with rheumatic pathology: oligoarticular juvenile arthritis in 2 patients; chronic recurrent multifocal osteomyelitis in 1 patient; acute rheumatic fever in 1 patient and two diseases, juvenile ankylosing spondylitis and Henoch-Schonlein hemorrhagic vasculitis, in 1 patient. In all the comorbid patients FMF diagnosis was verified by the detection of MEFV gene mutation: 3 homozygotes by M694V mutation, 2 compound heterozygotes M694V/V726A and M694V/M680I.
The comorbid patients included 2 boys and 3 girls. Four of these patients developed rheumatic diseases on the background of FMF and 1 patient developed FMF after the attack of acute rheumatic fever. The age of FMF onset in the comorbid patients was 1 to 11 years, mean age was 7.75 years. The onset of concomitant rheumatic diseases was at the age of 5 to 11 years, mean age was 9 years; the interval between the onset of FMF and rheumatic disease was 1 month to 9 years. Concomitant rheumatic pathology developed in two patients treated with colchicine. The therapy with TNF inhibitors (Etanercept) was administered to the two patients with FMF and juvenile arthritis.
Conclusions The patients with FMF in the Russian population quite often have concomitant rheumatic pathology. FMF may be combined with two rheumatic diseases in the same patient. Adequate colchicine therapy does not prevent the development of concomitant rheumatic disease.
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Disclosure of Interest None declared