Juvenile
polyarteritis nodosa (PAN) is a rare, necrotizing
vasculitis, primarily affecting small to medium-sized muscular arteries. Cardiac involvement amongst patients with PAN is uncommon and reports of coronary artery
aneurysms in juvenile PAN are exceedingly rare. We describe a 16 year old girl who presented with
fever,
arthritis and two giant coronary artery
aneurysms, initially diagnosed as atypical
Kawasaki disease and treated with
IVIG and
methylprednisolone. Her persistent
fevers,
arthritis, myalgias were refractory to treatment, and onset of a vasculitic
rash suggested an alternative diagnosis. Based on angiographic abnormalities, polymyalgia,
hypertension and skin involvement, this patient met criteria for juvenile PAN. She was treated with six months of intravenous
cyclophosphamide and high dose
corticosteroids for presumed PAN related coronary
vasculitis. Maintenance
therapy was continued with
azathioprine and the patient currently remains without evidence of active
vasculitis. She remains on anticoagulation for persistence of the
aneurysms. This case illustrates a rare and unusual presentation of giant coronary artery
aneurysms in the setting of juvenile PAN.