Acta paediatrica
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Clinical Trial
Clinical, laboratory and molecular characteristics of children with Familial Mediterranean Fever-associated vasculitis.
Familial Mediterranean Fever (FMF) is an autosomal recessive disease characterized by recurrent self-limited attacks of fever accompanied by peritonitis, pleuritis and arthritis. Approximately 5% of individuals with FMF have been reported to have Henoch-Schönlein purpura (HSP) and about 1% have polyarteritis nodosa (PAN). Protracted febrile myalgia is another vasculitis-associated clinical entity among patients with FMF. ⋯ In six children only one mutation was found and in three none of the studied mutations were identified. This study confirms that most children with FMF-associated vasculitis have identifiable mutations in the MEFV gene. Environmental and/or other genetic factors are possibly involved in the pathogenesis of vasculitis in FMF; elucidation of these mechanisms will help to understand pathogenesis of childhood vasculitides.