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DOI:10.2214/AJR.09.2623
AJR 2009; 193:W478-W481
© American Roentgen Ray Society

Clinical Observations

Coronary Abnormalities in Hyper-IgE Recurrent Infection Syndrome: Depiction at Coronary MDCT Angiography

Ahmed M. Gharib1, Roderic I. Pettigrew2, Abdalla Elagha1, Amy Hsu3, Pam Welch4, Steven M. Holland3 and Alexandra F. Freeman3

1 Department of Radiology, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Clinical Research Center, Bldg. 10, Rm. 3-5340, MSC 1263, 10 Center Dr., Bethesda, MD 20892.
2 National Institute of Biomedical Imaging and Bioengineering, National Institutes of Health, Bethesda, MD.
3 National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD.
4 SAIC-Frederick, National Cancer Institute-Frederick, Frederick, MD.

OBJECTIVE. Hyper-IgE recurrent infection syndrome (HIES or Job's syndrome) is a rare disorder affecting the immune system and connective tissues. The purpose of this study is to describe the coronary abnormalities in genetically confirmed HIES patients as depicted by coronary MDCT angiography (MDCTA).

CONCLUSION. Coronary MDCTA has provided an opportunity for noninvasive evaluation of the coronary arteries in patients with HIES. These coronary abnormalities vary from tortuosity to ectatic dilation and focal aneurysms of the coronary arteries. Such an evaluation has potential value in identifying new aspects of this disease and thereby providing better understanding of the pathophysiology of the disorder.

Keywords: coronary abnormalities • coronary aneurysms • ectatic coronaries • hyper-IgE recurrent infection syndrome (HIES or Job's syndrome) • coronary MDCT angiography


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