Coronary Artery Manifestation in Pediatric Takayasu’s Arteritis

Childhood onset Takayasu’s arteritis (TA) is a rare entity of large-vessel vasculitis that typically affects the aorta and its main branches and is otherwise observed in women between 20 and 30 years of age [Aeschlimann FA et al. Presentation and Disease Course of Childhood-Onset Versus Adult-Onset Takayasu Arteritis. Arthritis Rheumatol. 2019; 71 (2): 315– 323]. The etiology of the pathological inflammatory process that may lead to severe morbidity as a result of organ ischemia and chronic disease course is not well understood so far even though a genetic contribution to disease susceptibility is being increasingly recognized [Di Santo M, et al. Takayasu arteritis in paediatrics. Cardiol Young. 2018; 28 (3): 354–361]. In the early stage, diagnosis is frequently difficult and delayed because of nonspecific symptoms. The classification criteria of the updated EULAR recommendations for childhood vasculitides that include pathological imaging findings of the aorta and main branches achieve a high diagnostic accuracy, see table 1 [Ozen et al. EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides, Ann Rheum Dis 2006; 65: 936– 941]. Cardiovascular imaging is essential for diagnosis and disease monitoring with MRI including MR angiography (MRA) as the most relevant imaging modality. We present an exceptional case of childhood TA with disease onset in the left coronary artery and delayed diagnosis due to initially absent inflammatory changes in blood work and other vessel involvement.