Giant coronary artery aneurysms in juvenile polyarteritis nodosa: a case report

Juvenile polyarteritis nodosa (PAN) is a rare, necrotizing vasculitis, primarily affecting small to medium-sized muscular arteries [1]. Recent classification criteria from EULAR/PRES require histopathology of necrotizing vasculitis or angiographic abnormalities (aneurysm, stenosis, or occlusion of small-medium arteries), plus one of five of the following: skin involvement, myalgia or muscle tenderness, hypertension, peripheral neuropathy, or renal involvement, for diagnosis [2]. Cardiac involvement amongst patients with PAN is not common. Well described cardiac manifestations in adults with PAN include pericarditis, arrhythmia and valvular disease. There are infrequent reports of coronary arteritis, stenosis [3], dissection [4], and occasionally aneurysms [5] associated with PAN, however renal and gastrointestinal aneurysms are more common [6,7]. There is a paucity of literature regarding coronary artery manifestations in juvenile PAN, with limited information on demographics, clinical characteristics, treatment, and outcomes. Herein we describe a case of coronary artery aneurysms in an adolescent with juvenile PAN.This is a report of a 16 year old girl from St. Croix, U.S. Virgin Islands, who had been previously diagnosed with juvenile idiopathic arthritis (JIA) at 5 years of age, based on reports of fever, arthritis, and chronic uveitis. She received no systemic immunosuppressant medication and appeared to be in remission for several years. In December 2009 she presented with three days of sharp, substernal chest pain, two days of fever, and one day of bilateral hip pain causing difficulty ambulating. Physical exam revealed a tall, slender girl with mild nasal congestion. On cardiac exam she had a regular rate and rhythm, normal S1 and S2, and no appreciable murmur. Examination of her extremities revealed tenderness over her bilateral greater trochanters and bilateral knee effusions. Initial laboratory testing demonstrated white blood cell count of 5.7 k/μl, hemog