Increased Coronary Vessel Wall Thickness in Hyper IgE Syndrome Patients; Depiction by Magnetic Resonance Vessel Wall Imaging and Pathological Correction
Khaled Z. Abd-Elmoniem1, Nadine Z. Ramos1, Saami Yazdani2, Ahmed M. Ghanem1,3, Steven M. Holland4, Alexandra F. Freeman4, and Ahmed M Gharib1
1Biomedical and Metabolic Imaging Branch, NIDDK, Bethesda, MD, United States, 2University of Southern Alabama, Mobile, AL, United States, 3Electrical Engineering, Suez Canal University, Ismailia, Egypt, 4NIAID, Bethesda, MD, United States
In this study, coronary
wall MRI is used to assess the coronary wall thickness of patients with autosomal dominant hyper-IgE (AD-HIES) or Job's syndrome; a primary
immunodeficiency caused by mutations in STAT3. Supported by post-mortem histology, MRI coronary wall of AD-HIES patients was thicker than in healthy
subjects but comparable to CAD patients. These findings suggest that coronary
arteries in Job’s syndrome are affected with atherosclerosis, contrary to prior
beliefs and study findings. Direct histologic evaluation confirms the presence
of atherosclerosis with lack of needed supportive adventitial thickening and
elastic components. These findings suggest mechanisms for weakened vessel wall
that may lead to coronary dilation and aneurysm in AD-HIES.
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