ABSTRACT

Familial Mediterranean fever (FMF) is an autosomal recessive disease, cha-racterized by recurrent and self-limited attacks of fever, usually accompanied by polyserositis. Several vasculitic diseases such as Henoch-Schönlein purpura and polyarteritis nodosa (PAN) have been reported with increased frequency in patients with FMF. Although the pathogenesis is not clearly defined, it has been hypothesized that immune complexes may play a role in the association of the vasculitides and FMF. In this report we describe a 9-year-old boy admitted to our hospital with abdominal pain and fever. FMF was detected and colchicine treatment was given. Despite the treatment, his constitutional symptoms persis-ted. Due to the increased likelihood for the development of a vasculitic process in patients with FMF, angiography was performed because of a suspicion of PAN and angiography showed hepatic artery aneurysms. He responded well to pulse methylprednisolone and pulse cyclophosphamide therapy in addition to colchicine. We emphasize the rare association of FMF and PAN and insulated hepatic artery aneurysmal angiographic signs of PAN