Abstract
BACKGROUND: Coronary artery (CA) aneurysms in children are a rare but potentially life-threatening finding and are highly associated with Kawasaki disease (KD). CASE PRESENTATION: We describe a four-year-old female with a vasculitis and CA aneurysms. She had a prolonged course with recurrent fever and systemic inflammation several times upon discontinuation of steroid treatment. Due in part to the CA aneurysms, she initially was diagnosed with KD but due to the unusual clinical course, further evaluation was performed. Abdominal and chest MRI/A revealed diffuse aortitis suggestive of a large vessel vasculitis, specifically Takayasu arteritis. With treatment targeted for Takayasu arteritis, there was resolution of fever and inflammation and the CA aneurysms improved. CONCLUSIONS: This case demonstrates the utility in broadening the differential diagnosis in cases of presumed KD with CA involvement in which the clinical course is atypical for KD.