Introduction: Some necrotizing vasculitis may be associated with familial Mediterranean fever (FMF). We report a new case of polyarteritis nodosa (PAN) that preceded the diagnosis of FMF.
Observation: A young woman of Turkish origin had a long childhood history of inflammatory arthralgia and myalgia, leading to the provisional diagnosis of chronic juvenile arthritis, then, after a confirmative muscle biopsy, to the diagnosis of PAN, whose outcome remained benign. At the age of 19, she was diagnosed as having FMF on clinical and genetic grounds, and colchicine led to the regression of most symptoms.
Discussion: As with Henoch-Schönlein's purpura, PAN seems significantly associated with FMF. Its characteristics are a younger age at onset, more frequent peri-renal hematoma, overlap between classical PAN and micropolyangeitis, and overall better prognosis. In its muscular form, PAN is difficult to distinguish from protracted febrile myalgia, a recently described manifestation of FMF, in which pathological findings are poorly documented.
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