Abstract

Familial Mediterranean fever (FMF) is an autosomal recessively inherited autoinflammatory disorder that is characterized by recurrent attacks of fever and polyserositis. Approximately 90% of patients are diagnosed in childhood. Typical attacks last for 1-3 days and there is an attack-free period without any complaints. Most common symptoms are fever and abdominal pain. Other common symptoms are chest pain and arthritis due to pleural and synovial involvement. Rash, eye involvement, epididymo-orchitis and glomerulonephritis are rare manifestations of FMF. Herein, we present a case of FMF whose initial symptoms were fever, fatigue and weight loss with high acute phase reactants and abdominal lympadenopathy that was mimicking a malignant process

Keywords: Familial Mediterranean fever, Childhood, Lymphadenopathy

References

  1. Ben-Chetrit E, Touitou I. Familial Mediterranean fever in the world. Arthritis Rheum 2009; 61:1447–53.
  2. Soriano A, Manna R. Familial Mediterranean fever: New phenotypes. Autoimmun Rev 2012;12:31-7
  3. Yalçinkaya F, Cakar N, Misirlioğlu M, Tümer N, Akar N, Tekin M, et al. Genotype-phenotype correlation in a large group of Turkish patient with familial mediterranean fever: Evidence for mutation independent amyloidosis. Rheumatology (Oxford) 2000;39:67–72
  4. Tunca M, Akar S, Onen F, Ozdogan H, Kasapcopur O, Yalcinkaya F, et al. Turkish FMF Study Group. Familial Mediterranean fever (FMF) in Turkey: Results of a nationwide multicenter study. Medicine 2005;84:1-11
  5. Goldfinger SE. Colchicine for familial Mediterranean fever. N Engl J Med 1972;287:1302.
  6. Ayata A. Çocukluk çağında lenfadenopatiler. SDÜ Tıp Fakültesi Dergisi 2004;11:26-29
  7. Çiftçi E, Özdemir H, İncesoy S, İnce E, Doğru Ü. Periyodik ateş, aftöz stomatit, farenjit ve servikal adenit (PFAPA) sendromlu bir olgu. Türk Pediatri Arşivi 2004;39:36-40.
  8. Genç B. Çocukluk çağında lenfadenopatilere yaklaşım. Journal of Current Pediatrics 2014;1:6-12
  9. Tamir N, Langevitz P, Zemer D, Pras E, Shinar Y, Padeh S, et al. Late-onset familial Mediterranean fever (FMF): A subset with distinct clinical, demographic, and molecular genetic characteristics. Am J Med Genet 1999;87:30-5
  10. Shohat M, Halpern GJ. Familial Mediterranean fever-a review. Genet Med 2011;13:487-89
  11. Alonso R, Cisterò-Bahima A, Enrique E, San Miguel-Moncín MM. Recurrent urticaria as a rare manifestation of familial Mediterranean fever. J Investig Allergol Clin Immunol 2002;12:60-1.
  12. Yazici H, Pazarli H. Eye involvement in a patient with familial Mediterranean fever. J Rheumatol 1982;9:644
  13. Karachaliou I, Karachalios G, Charalabopoulos A, Charalabopoulos K. Meningitis associated with familial Mediterranean fever. Int J Clin Pract Suppl 2005;147:60–1.
  14. Kasifoglu T, Cansu DU, Korkmaz C. Frequency of abdominal sur- gery in patients with familial Mediterranean fever. Intern Med 2009;48:523-6.
  15. Aharoni D, Hiller N, Hadas-Halpern I. Familial Mediterranean fever: Abdominal imaging findings in 139 patients and review of the literature. Abdom Imaging 2000;25;297-300.
  16. Zissin R, Rathaus V, Gayer G, Shapiro-Feinberg M, Hertz M. CT findings in patients with familial Mediterranean fever during an acute abdominal attack. Br J Radiol 2003;76:22-5.
  17. Gattorno M, Martini A. Beyond the NRLP3 inflammasome: Autoinflammatory diseases reach adolescence. Arthritis Rheum 2013;65:1137-47.

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How to cite

1.
Keskindemirci G, Ayaz NA, Tuğcu D, Çakan M, Aydoğan G, Er A, et al. A Rare Cause of Lymphadenopathy in Children: Autoinflammatory Disease - Familial Mediterranean Fever. Turk J Pediatr Dis [Internet]. 2016 Dec. 1 [cited 2025 May 24];10(4):274-6. Available from: https://turkjpediatrdis.org/article/view/369