Coronary Artery Aneurysms as a Feature of Granulomatosis With Polyangiitis

Granulomatosis with polyangiitis is an antineutrophil cytoplasmic antibody-associated vasculitis that primarily affects small vessels. The disease typically affects the respiratory tract and kidneys but has also been known to involve the gastrointestinal tract, genitourinary tract, thyroid, and liver. Cardiac involvement is rare. Coronary artery aneurysms (CAAs) are an extremely uncommon finding, with only 1 reported case in an adult patient and no pediatric cases reported to date. Here, we report the unique case of a child with granulomatosis with polyangiitis who initially presented with fever of unknown origin and pulmonary and renal symptoms with no cardiac complaints. An echocardiogram revealed severe bilateral fusiform CAAs. Because of the high risk of mortality posed by the severity of her renal and cardiac disease, the patient was managed with intensive induction immunosuppression with steroids, rituximab, and cyclophosphamide. She is maintained on steroids, rituximab, aspirin, and warfarin with improved renal function but no change in her CAAs.