Polyarteritis nodosa involving the hepatobiliary system in an eight-year-old girl with a previous diagnosis of familial Mediterranean fever

Polyarteritis nodosa (PAN) is a vasculitis of small- and medium-sized muscu lar arteries with deposition of immune complex in the vessel wall. Although gastrointestinal involvement is common, the symptomatic involvement of the hepatobiliary system is rare. An eight-year old female patient with a prev ious diagnosis of familial Mediterranean fever (FMF) was hospitalized for r ight upper quadrant pain and fever. The thickened gall bladder wall by ultr asonography, called for exploration. Histopathological evaluations of the l iver biopsy and gall bladder revealed PAN. Corticosteroid therapy was initi ated and the patient recovered fully. This case represents one of the rares t forms of PAN in childhood.