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Erschienen in:

01.06.2011

Coronary Artery Abnormalities in Hyper-IgE Syndrome

verfasst von: Alexandra F. Freeman, Elizabeth Mannino Avila, Pamela A. Shaw, Joie Davis, Amy P. Hsu, Pamela Welch, Jatin R. Matta, Colleen Hadigan, Roderic I. Pettigrew, Steven M. Holland, Ahmed M. Gharib

Erschienen in: Journal of Clinical Immunology | Ausgabe 3/2011

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Abstract

Objective

Hyper-IgE syndrome (HIES) is a rare primary immunodeficiency caused by autosomal dominant STAT3 mutations resulting in recurrent infections and connective tissue abnormalities. Coronary artery abnormalities have been reported infrequently. We aimed to determine the frequency and characteristics of coronary artery abnormalities.

Design

STAT3-mutated HIES patients (n = 38), ranging in age from 8 to 57 years, underwent coronary artery imaging by computed tomography or magnetic resonance imaging. Images were evaluated for tortuosity, dilation, and aneurysm. Charts were reviewed for cardiac risk factors. To allow blinded image interpretation, an age- and gender-matched non-HIES group was also evaluated (n = 33).

Results

Coronary artery tortuosity or dilation occurred in 70% of HIES patients, with aneurysms present in 37%, incidences much higher than in the literature and in our non-HIES group, in which 21% had tortuosity or dilation and 3% had aneurysms. Hypertension was more common in the HIES group than in the general population and was associated with vessel abnormalities. Atherosclerosis was uncommon and mild.

Conclusions

Coronary artery aneurysms and tortuosity are common in HIES, despite a paucity of atherosclerosis, suggesting that STAT3 plays an integral role in human vascular remodeling and atherosclerosis.

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Titel: Coronary Artery Abnormalities in Hyper-IgE Syndrome
verfasst von: Alexandra F. Freeman
Elizabeth Mannino Avila
Pamela A. Shaw
Joie Davis
Amy P. Hsu
Pamela Welch
Jatin R. Matta
Colleen Hadigan
Roderic I. Pettigrew
Steven M. Holland
Ahmed M. Gharib
Publikationsdatum: 01.06.2011
Verlag: Springer US
Erschienen in: Journal of Clinical Immunology / Ausgabe 3/2011
Print ISSN: 0271-9142
Elektronische ISSN: 1573-2592
DOI: https://doi.org/10.1007/s10875-011-9515-9

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