Polyarteritis nodosa presenting as temporal arteritis in a 9-year-old child

A 9-year-old Haitian girl presented initially with monocular blindness and an isolated temporal arteritis, confirmed by angiographic studies and tempo ral artery biopsy findings. CT and MR studies of the intracranial circulati on showed only an enlarged, dense superficial temporal artery. Systemic wor kup revealed a mildly elevated erythrocyte sedimentation rate, mild changes in white and fed blood cells, and a remote history of sensorineural hearin g loss. Pathologic examination of the biopsy specimen narrowed the differen tial diagnosis to giant cell temporal arteritis and polyarteritis nodosa. T reatment with corticosteroids alone failed, and the child returned 1 month later with severe systemic illness and encephalopathy, MR studies showed mu ltiple cortical and subcortical foci of increased T2 signal, and gyriform e nhancement on T1-weighted images. Renal and mesenteric arteriograms showed innumerable tiny aneurysms at branch points in small and medium-sized vesse ls, typical of polyarteritis nodosa, We found no previous reports of this i nitial presentation in the pediatric population for either polyarteritis no dosa or giant cell temporal arteritis.