Protracted Febrile Myalgia in a Child as the Presenting Sign of Familial Mediterranean Fever: Case Report and Review of the Literature Department of Pediatric Nephrology, Gülhane Military Medical Academy, Ankara, Turkey
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چکیده
Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent self-limited attacks of fever accompanied by peritonitis, pleuritis, and arthritis. The disease is predominantly seen in Mediterranean populations (Jews, Armenians, Arabs, Turks). Several types of vasculitis are associated with FMF: Polyarteritis Nodosa (PAN) and Henoch-Schonlein Purpura (HSP) are the best delineated ones. Protracted febrile myalgia (PFM) is another rare form of vasculitic disease which is an uncomman dramatic manifestation of FMF. PFM may occur despite colchicine therapy and requires treatment with corticosteroids. PFM, fi rst described by Langevitz et al. (1) in 14 FMF patients in 1994, is characterized by severe paralyzing myalgia and high fever, sometimes accompanied by abdominal pain, diarrhea, and arthritis/arthralgia, and in a few patients by transient vasculitic purpura mimicking HSP. The episode lasts for 4-6 weeks, except in those patients treated with corticosteroids. High ESR, hyperglobulinemia, normal CPK, and subtle nonspecifi c infl ammatory myopathic changes on EMG are the other characteristics (1, 2). In this report, we present a child with PFM as the sole feature preceding the diagnosis of FMF and draw attention to the PFM for the diagnosis of FMF even the patient does not fulfi ll the criteria for the clinical diagnosis.
منابع مشابه
Familial Mediterranean Fever: Review of Literature and Report of Two Cases
Familial Mediterranean fever, an autosomal recessive disorder, is a member of the periodic fever syndromes, and considered to be the most common cause of recurrent febrile episodes in children. It is important to understand the disorder as familial Mediterranean fever falls on a spectrum of various presentations; the recurrent episodes of familial Mediterranean fever may be so severe that the q...
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