First, let’s meet the patient.
A 7-year-old girl with recently diagnosed hypertension, ulcerative colitis, and a history of Henoch-Schönlein purpura presented to the emergency department with headache, chest pain, abdominal pain, and an elevated blood pressure of 173/121 mm Hg in her right upper extremity.
Physical examination findings were significant for weak left carotid, brachial, and radial pulses and bilateral carotid bruits. Renal ultrasonography with Doppler was performed, the results of which were concerning for renal artery stenosis, and electrocardiography findings suggested ventricular hypertrophy.
Multiple antihypertensive agents were successively started, resulting in a slow reduction but not normalization of her blood pressure. An echocardiogram showed normal biventricular function, mild aortic root dilation, aortic insufficiency, and a diffusely dilated left coronary artery. The erythrocyte sedimentation rate (ESR) and the C-reactive protein (CRP) level were found to be elevated to 24 mm/h and 27 mg/L, respectively, raising concern for a diffuse inflammatory process.
Additional angiography of her brain, chest, and abdominal vasculature was performed, revealing significant narrowing of the left common, external, and internal carotids, and of the bilateral subclavian, hepatic, splenic, and renal arteries; celiac axis narrowing; asymmetric kidney size; and diffuse thickening of the aorta from the heart through the abdomen, with a bright wall signal (Figure).
