Adult-onset familial mediterranean fever: case reports with literature review
https://doi.org/10.18705/2782-3806-2024-4-5-452-457
EDN: RMJMYU
Abstract
Familial Mediterranean Fever (FMF) is a rare monogenic autoinflammatory disease that typically manifests in early childhood. The disease is characterized by its prevalence within a specific ethnic group. Nevertheless, FMF can also manifest in later stages of life among individuals with or without an ethnic background. We present two case reports of FMF patients, where the onset of the disease occurred in the third decade of life. These cases emphasize the importance of including monogenic autoinflammatory diseases in the differential diagnosis of adults presenting with fever of unknown origin and exhibiting typical FMF symptoms regardless of ethnicity.
Keywords
About the Authors
V. Yu. MyachikovaRussian Federation
Maslyanskiy Alexey L. - PhD, MD, Rheumatologist, Professor of the Department of Faculty Therapy with the clinic of Medical Education; Head of the department “Research Laboratory of Rheumatology and immunopathology”, Almazov NMRC; Professor of the Scientific, Clinical and Educational Centre of Gastroenterology and Hepatology, Saint-Petersburg SU.
Akkuratova str., 2, Saint Petersburg, 197341
K. A. Aksenova
Russian Federation
Aksenova Ksenia A. - 6th year student of the Institute of Medical Education.
Saint-Petersburg
A. A. Khodot
Russian Federation
Khodot Anna A. - 6th year student of the Institute of Medical Education.
Saint-Petersburg
A. L. Maslyanskiy
Russian Federation
Myachikova Valentina Yu. - PhD, Rheumatologist, Professor assistant of the Department of Faculty Therapy with the clinic of Medical Education; a researcher World-Class Research Centre for Personalized Medicine, Rare and Genetically Determined Diseases, Research Laboratory of Autoimmune and Autoinflammatory Diseases.
Saint-Petersburg
References
1. Association of Pediatric Rheumatologists, Association of Medical Geneticists. Clinical recommendations: Familial Mediterranean Fever, 2023. In Russian
2. Lancieri M, Bustaffa M, Palmeri S, et al. An Update on Familial Mediterranean Fever. Int. J. Mol. Sci. 2023, 24, 9584. https://doi.org/10.3390/ijms24119584
3. Aydin O, Egeli BH, Ozdogan H, et al. Late-onset familial mediterranean fever: single-center experience and literature review. Intern Emerg Med. 2022 Aug;17(5):1301–1306. DOI: 10.1007/s11739-021-02912-8. Epub 2022 Jan 21. Erratum in: Intern Emerg Med. 2022 Mar 4;: PMID: 35061158.
4. Gattorno M, Hofer M, Federici S, et al. For the Eurofever Registry and the Paediatric Rheumatology International Trials Organisation (PRINTO). Classification criteria for autoinflammatory recurrent fevers. Annals of the Rheumatic Diseases 2019;78:1025–1032.
5. Aydin F, Özçakar ZB, Çakar N, et al. Sacroiliitis in Children With Familial Mediterranean Fever. J Clin Rheumatol. 2019 Mar;25(2):69–73. DOI: 10.1097/RHU.0000000000000770. PMID: 29596210.
6. Ozdogan H, Ugurlu S. Familial Mediterranean Fever. Presse Med. 2019 Feb;48(1 Pt 2): e61–e76. DOI: 10.1016/j.lpm.2018.08.014. Epub 2019 Jan 25. PMID: 30686512.
7. Fedorov EU, Salugina CO. Familial Mediterranean fever (periodic illness): a story or a real problem. Modern rheumatology. 2018;12(3):61–69. In Russian
8. Eliseeva LN, Bocharnikova MI. Three cases of late diagnosis of periodic illness. The South Russian Journal of Therapeutic Practice. 2021;2(2):94-99. DOI:10.21886/2712-8156-2021-2-2-94-99 In Russian
9. Ozen S, Demirkaya E, Erer B, et al. EULAR recommendations for the management of familial Mediterranean fever. Annals of the Rheumatic Diseases. 2016;75:644–651.
10. Sohar E, Gafni J, Pras M, Heller H. Familial Mediterranean Fever. A Survey of 470 Cases and Review of the Literature. Am. J. Med. 1967; 43: 227–53.
11. Tufan A, Lachmann HJ. Familial Mediterranean fever, from pathogenesis to treatment: a contemporary review. Turk J Med Sci. 2020 Nov 3;50(SI-2):1591–1610. DOI: 10.3906/sag-2008-11. PMID: 32806879; PMCID: PMC7672358.
Review
For citations:
Myachikova V.Yu., Aksenova K.A., Khodot A.A., Maslyanskiy A.L. Adult-onset familial mediterranean fever: case reports with literature review. Russian Journal for Personalized Medicine. 2024;4(5):452-457. (In Russ.) https://doi.org/10.18705/2782-3806-2024-4-5-452-457. EDN: RMJMYU