Systemic and pulmonary artery aneurysms in incomplete Kawasaki disease

Kawasaki disease (KD) is a systemic vasculitis that can cause coronary artery aneurysms (CAA) in up to 25% if left untreated. Rarely, aneurysms of systemic arteries of all sizes have been reported to occur. The incidence of systemic artery aneurysms (SAA) with typical KD can be as high as 2.2%. Incomplete KD with SAA is not well described. We report a case of a 12-year-old boy with incomplete KD, giant CAA, diffuse SAA, and pulmonary artery aneurysms (PAA). The patient presented with fever, malaise, abdominal pain, maculopapular, rash and cervical lymphadenopathy. Echocardiogram showed multiple giant CAA. By angiography, diffuse ectasia and aneurysms of most medium-sized arteries throughout the body were noted including the lobar pulmonary artery branches. Incomplete KD was suspected. The patient was treated with intravenous gammaglobulin, methylprednisolone, and high-dose aspirin. Due to the systemic vasculitis, he also received cyclophosphamide. The patient's clinical symptoms improved. Anticoagulation with warfarin was maintained. The patient remains asymptomatic three years later but with progressive CAA and stable SAA. The PAA caused no symptoms and resolved after the acute phase. Incomplete KD can manifest with CAA and SAA. This is the first report of PAA associated with KD. Surveillance for PAA in KD must be considered. <Learning objective: A high level of clinical suspicion is required to diagnose KD in adolescent patients as it can manifest in an atypical fashion. Incomplete KD can manifest with giant CAA and SAA. Pulmonary artery aneurysms (PAA) have not been previously described in cases of KD. This is the first case report of atypical KD with PAA. Imaging of pulmonary arteries and its branches should be considered in patients with atypical KD with CAA and SAA.>.