Hacer İlbilge Ertoy KARAGÖL, Cüneyt KARAGÖL, Harun PERU, Ahmet Midhat ELMACI

Uzamış febril myalji sendromu ile ortaya çıkan ailesel Akdeniz ateşi olgusu

Ailesel Akdeniz ateşi herediter periyodik ateş sendromlarının en sık görülenidir. Hastalığın seyrinde vaskülitik sendromlara daha sıklıkla rastlanmaktadır. Uzamış febril myalji sendromu, ailesel Akdeniz ateşine özel klinik bir tablodur. Bu sendromun en karakteristik bulguları şiddetli kas ağrısı, uzamış yüksek ateş, artmış eritrosit sedimentasyon hızı, hipergamaglobülinemi, normal kreatin fosfokinaz ve elektromyografideki non-spesifik inflamatuar myopatik değişikliklerdir. Bu yazıda, uzamış febril myalji sendromu ile ortaya çıkan ve steroid tedavisine dramatik yanıt veren ailesel Akdeniz ateşi olgusu bildirilmiştir.

A case of familial Mediterranean fever presenting with protracted febrile myalgia syndrome

Familial Mediterranean fever is the most common in the hereditary periodic fever syndromes. The frequency of vasculitis syndromes are high in familial Mediterranean fever population. Protracted febrile myalgia syndrome is an familial Mediterranean fever-specific manifestation. This syndrome is characterized by severe myalgia, protracted high fever, increased erythrocyte sedimentation rate, hypergammaglobulinemia, normal creatine phosphokinase, and nonspecific inflammatory myopathic changes on electromyography. In this letter, we reported a case of familial Mediterranean fever presenting with protracted febrile myalgia syndrome as dramatically responsive steroid therapy.

PDF

___

1. Ben-Chetrit E, Levy M. Familial Mediterranean fever. Lancet 1998;351:659-664.
2. Livneh A, Pras M, Padeh S, et al. Criteria for the diagnosis of familial Mediterranean fever. Arthritis Rheum 1997;40:1879-1885.
3. Sohar E, Gafhi J, Pras M, et al. Familial Mediterranean fever: A survey of 470 cases and review of the literature. Am J Med 1967;43:227-253.
4. Tekin M, Yalcinkaya F, Tumer N, et al. Clinical, laboratory and molecular characteristics of children with familial Mediterranean fever-associated vasculitis. Acta Paediatr 2000;89:177-182.
5. Ozdogan H, Arisoy N, Kasapcopur O, et al. Vasculitis in familial Mediterranean fever. J Rheumotol 1997;24:323-327.
6. Langevitz P, Zemer D, Livneh A, et al. Protracted febrile myalgia in patients with familial Mediterranean fever. J Rheumatol 1994;21:1708-1709.
7. Livneh A, Langevitz P, Zemer D, et al. Criteria for the diagnosis of familial Mediterranean fever. Arthritis Rheum 1997;40:1879-1885.
8. Odabas AR, Cetinkaya R, Selcuk Y, et al. Familial Mediterranean fever. South Med 2002;95:1400-1403.
9. Özdoğan H, Kasapcopur O, Arısoy N. Ailesel Akdeniz Ateşi. Klinik Gelişim Dergisi. 2006;19:68-73.
10. Odabas AR, Cetinkaya R, Selcuk Y, et al. Severe and prolonged febrile myalgia in familial Mediterranean fever. Scand J Rheumatol 2000;29:394-395.
11. Majeed HA, Rawashdeh M, el-Shanti H, et al Familial Mediterranean fever in children: the expanded clinical profile. QJM 1999;92:309-318.
12.Livneh A, Langevitz P. Diagnostic and treatment concerns in familial Mediterranean fever. Baillieres Best Pract Res Clin Rheumatol 2000;14:477-498.
13. Shapiro TR, Ehrenfeld EN. Recurrent polyserositis (periodic disease, familial Mediterranean fever) in children. Pediatrics 1962;30:443-449.
14. Tekin M, Yalcinkaya F, Tumer N, et al. Familial Mediterranean fever and acute rheumatic fever: A pathogenetic relationship? Clin Rheumatol 1999;18:446-449.
15. Soylu A, Kasap B, Türkmen M, et al. Febrile myalgia syndrome in familial Mediterranean fever. J Clin Rheumatol 2006;12:93-96