Coronary abnormalities in hyper-IgE recurrent infection syndrome: depiction at coronary MDCT angiography.
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).


CONCLUSIONS
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.
Gharib
Ahmed M
AM
Department of Radiology, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Clinical Research Center, Bethesda, MD 20892, USA. agharib@mail.nih.gov
Pettigrew
Roderic I
RI
Elagha
Abdalla
A
Hsu
Amy
A
Welch
Pam
P
Holland
Steven M
SM
Freeman
Alexandra F
AF
D000328:Adult; D003287:Contrast Media; D017023:Coronary Angiography / Q000379:methods*; D003327:Coronary Disease / Q000000981:diagnostic imaging*; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D021621:Imaging, Three-Dimensional; D007073:Immunoglobulin E / Q000097:blood; D007479:Iopamidol; D007589:Job Syndrome / Q000000981:diagnostic imaging*; D008297:Male; D008875:Middle Aged; D011857:Radiographic Image Interpretation, Computer-Assisted; D012008:Recurrence; D014057:Tomography, X-Ray Computed / Q000379:methods*
D003287:Contrast Media; D007073:Immunoglobulin E; D007479:Iopamidol