Childhood-onset Takayasu Arteritis

Abstract

Childhood-onset Takayasu Arteritis (cTAK) is a rare, large-vessel type of vasculitis seen in children, mainly affecting the aorta and its major branches. Clinical manifestations are often severe and arise as a result of systemic and local inflammation, along with end-organ ischemia. Disease flares are common and the disease burden is high, with a significant rate of morbidity and mortality. Recent advances in understanding the underlying disease pathobiology resulted in the use of pathway-targeting agents, such as TNF- or IL-6 inhibitors with improved disease control. Nonetheless, the prognosis often remains guarded and the accrued damage is significant. This review aims at summarizing the recent evidence and observations regarding this condition, with a focus on pediatric publications.