Abstract
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.
Highlights
Juvenile polyarteritis nodosa (PAN) is a rare, necrotizing vasculitis, primarily affecting small to medium-sized muscular arteries [1]
Given that coronary artery aneurysms develop in 17% of untreated Kawasaki disease (KD), it is unsurprising that PAN is mistaken for KD [13]
Coronary aneurysms occur in diseases other than KD, and clinicians should have a higher index of suspicion for other pediatric vasculitides
Summary
Juvenile polyarteritis nodosa (PAN) is a rare, necrotizing vasculitis, primarily affecting small to medium-sized muscular arteries [1]. Due to a prolonged course of 8 days of fevers, the presence of the coronary aneurysms and a concern for atypical Kawasaki disease (KD), the patient was started on aspirin and received two courses of IVIG She was started on anticoagulation with warfarin. Two weeks after her rash appeared, she developed persistent hypertension, requiring treatment with amlodipine This patient met criteria for juvenile PAN based on angiographic abnormalities and myalgias. The patient had a single photon emission computed tomography (SPECT) myocardial perfusion scan with a Tc-99 m Sestamibi injection to assess for possible cardiac ischemia which may present during exertion During the test, she had no chest pain, and there were no electrocardiogram changes suggestive of ischemia. Acute phase reactants have normalized and the patient remains with no evidence of active vasculitis She was started on azathioprine for maintenance therapy and is slowly being tapered off of corticosteroids.
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