Great Vessels of Children: Takayasu's Arteritis

Takayasu's arteritis (TA) is a granulomatous, large vessel vasculitis affecting primarily the aorta and its main branches. It is characterized by inflammation in the blood vessel wall, leading to either luminal occlusion or dilatation with aneurysm formation. The etiology of TA is unknown, but there seems to be a strong role for cell-mediated autoimmunity in the pathogenesis of this disease. TA most commonly presents in young women in their second and third decades of life, but has been reported in children as young as age 2 years. The symptoms can range from vague systemic complaints to catastrophic stroke. Angiography remains the gold standard for diagnosis, although computed tomography angiography and magnetic resonance angiography have been used as well. Corticosteroids are first-line therapy, with various cytotoxic medications being used in refractory disease. Biologic agents targeting cytokines that are involved in disease pathogenesis have also been used successfully. In this article, we describe a patient with TA who responded to therapy with infliximab, an inhibitor of tumor necrosis factor.