AB1472 THE PECULIARITIES OF THE CLINICAL MANIFESTATIONS OF FAMILIAL MEDITERRANEAN FEVER ACCOMPANIED WITH LUPUS-LIKE SYNDROME

نویسندگان

چکیده

Background The most common autoinflammatory disease which occurs in Armenian ethnos is familial Mediterranean fever (FMF). FMF a hereditary disorder characterized by recurrent attacks of and serosal inflammation. main clinical manifestations are fever, abdominal, chest joint pain, erysipelas-like skin lesion but also has lot other rare systemic manifestations. Sometimes it difficult to distinguish if the patient only or overlap rheumatological diseases: lupus erythematosus (SLE), vasculitis, spondyloarthropathy, etc. Objectives purpose study reveal non-classic FMF, analyze compare course these cases. Methods 100 patients with genetically confirmed who fulfilled Tel-Hashomer classification criteria were included this study. Molecular-genetic detection 26 MEFV mutations more among Armenians was carried out. Only 13 from had like lupus, so we continued investigation determine ANA, anti-dsDNA antibodies, positive. All lupus-like syndrome diagnostic SLE ACR. Comparison peculiarity done between - both (group I) II). Results There 11 female 2 male first group. mean age 40,06±2,46. 15.4% affected syndrome/SLE parallelly 30.8% cases onset prior presentation FMF. They all such as erythema (92.3%), livedo reticularis (69.2%), photosensitivity Raynaud’s phenomenon (23.07%). Though frequency I group than II group, high temperature 39-40 C° viewed 23% 46% duration attack days respectively 1-3 1-5. We noticed that thoracalgia monoarthritis less frequently II, 7.7% respectively. difference presented below (Table 1). prevalent mutation gene groups M694V- 19 (73%), 3 (11,5%) homozygote 23 (88,5%) heterozygote. Table 1. Clinical (FMF coexisting SLE)% (only FMF)% Abdominalgia 92,3 76,9 Thoracalgia 69,2 Arthralgia Monoarthritis 53,8 Pleuritis Pericarditis 15,4 7,6 Conclusion In conclusion, diseases have manifestations: arthralgia, arthritis, involvement, pleuritis, pericarditis. milder: lower temperature, shorter days; appear frequently. Further investigations needed co-exciting rheumatic diseases. References [1]B. Schreiber, H. Lachmann, C. Mackworth-Young. Possible Caucasian erythematosus. Lupus, 2008;17(8):752-3. doi: 10.1177/0961203308089449. [2]T. Klein, Sh. Tiosano, Y. Gilboa, Shoenfeld, A. Cohen, Amital. coexistence (FMF) (SLE) A cross sectional 2021;30(7):1094-1099. 10.1177/09612033211004726. Acknowledgements: NIL. Disclosure Interests None Declared.

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ژورنال

عنوان ژورنال: Annals of the Rheumatic Diseases

سال: 2023

ISSN: ['1468-2060', '0003-4967']

DOI: https://doi.org/10.1136/annrheumdis-2023-eular.6158