Coronary atherosclerosis and dilation in hyper IgE syndrome patients: depiction by magnetic resonance vessel wall imaging and pathological correlation

KZ Abd-Elmoniem, N Ramos, SK Yazdani, AM Ghanem… - Atherosclerosis, 2017 - Elsevier
Background and aims Autosomal dominant hyper-IgE (AD-HIES) is a primary
immunodeficiency caused by mutations in STAT3. Elevated levels of IgE, an ineffective
immune response, connective tissue abnormalities, and coronary arterial dilation and
tortuosity characterize AD-HIES. To date, coronary artery evaluation in AD-HIES patients has
been limited to lumenography measurements. Direct in vivo coronary vessel wall (VW)
imaging may allow for better interrogation of coronary vessel abnormalities. The goal of this …