Highlights
- •Subclinical coronary atherosclerosis is evident on MR imaging in autosomal dominant hyper-IgE (AD-HIES) patients.
- •Histological evaluation confirms the presence of coronary atherosclerosis in AD-HIES.
- •There is paucity of supportive coronary adventitial thickening and elastic component.
- •Atherosclerosis without supportive structures may explain AD-HIES coronary dilation.
Abstract
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 prospective study was to evaluate the coronary
VW of AD-HIES patients using Magnetic Resonance Imaging (MRI) and histology. VW image
findings were compared in healthy subjects and subjects with coronary atherosclerotic
disease (CAD).
Methods
A total of 28 subjects (10 with AD-HIES, 8 healthy, 10 with CAD) were studied by coronary
VW MRI imaging. Additionally, a post-mortem coronary artery from one VW imaged AD-HIES
patient was examined.
Results
Coronary VW in AD-HIES was thicker than in healthy controls but not significantly
different from VW thickness in CAD subjects. AD-HIES coronaries showed increased VW
area compared to healthy controls and CAD subjects. On histology, the AD-HIES coronary
artery had findings consistent with atherosclerotic plaque, but had minimal luminal
narrowing, deficient adventitia thickening and absence of both internal and external
elastic laminae.
Conclusions
This is the first study to demonstrate subclinical coronary atherosclerosis in AD-HIES
patients on VW imaging by MRI. Histologic evaluation confirmed the presence of atherosclerosis
with lack of supportive adventitial thickening and elastic components. These findings
suggest mechanisms for coronary dilation in AD-HIES and thereby help direct clinical
management.
Keywords
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References
- Dominant-negative mutations in the DNA-binding domain of STAT3 cause hyper-IgE syndrome.Nature. 2007; 448: 1058-1062
- Hyper-IgE syndrome update.Ann. N. Y. Acad. Sci. 2012; 1250: 25-32
- Coronary artery abnormalities in Hyper-IgE syndrome.J. Clin. Immunol. 2011; 31: 338-345
- Frequent and widespread vascular abnormalities in human signal transducer and activator of transcription 3 deficiency.Circ. Cardiovasc Genet. 2012; 5: 25-34
- Coronary artery aneurysm: a review.Clin. Cardiol. 2006; 29: 439-443
- Pathophysiology of acute coronary syndrome.Curr. Atheroscler. Rep. 2014; 16: 401
- Compensatory enlargement of human atherosclerotic coronary arteries.N. Engl. J. Med. 1987; 316: 1371-1375
- Coronary abnormalities in hyper-IgE recurrent infection syndrome: depiction at coronary MDCT angiography, AJR.Am. J. Roentgenol. 2009; 193: W478-W481
- Coronary artery aneurysms in patients with hyper IgE recurrent infection syndrome.Clin. Immunol. 2007; 122: 255-258
- Increased coronary vessel wall thickness in HIV-infected young adults.Clin. Infect. Dis. 2014; 59: 1779-1786
- Coronary vessel wall 3-T MR imaging with time-resolved acquisition of phase-sensitive dual inversion-recovery (TRAPD) technique: initial results in patients with risk factors for coronary artery disease.Radiology. 2012; 265: 715-723
- Noninvasive coronary vessel wall and plaque imaging with magnetic resonance imaging.Circulation. 2000; 102: 2582-2587
- Feasibility of coronary artery wall thickening assessment in asymptomatic coronary artery disease using phase-sensitive dual-inversion recovery MRI at 3T.Magn. Reson. imaging. 2013; 31: 1051-1058
- Phase-sensitive black-blood coronary vessel wall imaging, Magnetic resonance in medicine.Off. J. Soc. Magn. Reson. Med./Soc. Magn. Reson. Med. 2010; 63: 1021-1030
- Di Carli M.F. Kwong K.F. Novel Techniques for Imaging the Heart: Cardiac MR and CT. American Heart Association Willey-Blackwell, Dallas2008: 238
- Arterial wall imaging: evaluation with 16-section multidetector CT in blood vessel phantoms and ex vivo coronary arteries.Radiology. 2006; 240: 708-716
- Automated quantification of coronary plaque with computed tomography: comparison with intravascular ultrasound using a dedicated registration algorithm for fusion-based quantification.Eur. Heart J. 2012; 33: 1007-1016
- Double-oblique free-breathing high resolution three-dimensional coronary magnetic resonance angiography.J. Am. Coll. Cardiol. 1999; 34: 524-531
- Simultaneous spatial and spectral selective excitation, Magnetic resonance in medicine.Off. J. Soc. Magn. Reson. Med./Soc. Magn. Reson. Med. 1990; 15: 287-304
- Novel real-time R-wave detection algorithm based on the vectorcardiogram for accurate gated magnetic resonance acquisitions, Magnetic resonance in medicine.Off. J. Soc. Magn. Reson. Med./Soc. Magn. Reson. Med. 1999; 42: 361-370
- Prospective navigator correction of image position for coronary MR angiography.Radiology. 1997; 203: 733-736
- Accuracy of 16-row multidetector computed tomography for the assessment of coronary artery stenosis.Jama. 2006; 296: 403-411
- Detection of calcified and noncalcified coronary atherosclerotic plaque by contrast-enhanced, submillimeter multidetector spiral computed tomography: a segment-based comparison with intravascular ultrasound.Circulation. 2004; 109: 14-17
- Initial evaluation of coronary images from 320-detector row computed tomography.Int. J. Cardiovasc. imaging. 2008; 24: 535-546
- Mathematical modeling of coupled drug and drug-encapsulated nanoparticle transport in patient-specific coronary artery walls.Comput. Mech. 2011; 49: 213-242
- Lung parenchyma surgery in autosomal dominant hyper-IgE syndrome.J. Clin. Immunol. 2013; 33: 896-902
- Plasma metalloproteinase levels are dysregulated in signal transducer and activator of transcription 3 mutated hyper-IgE syndrome.J. Allergy Clin. Immunol. 2011; 128: 1124-1127
- The association of genetic variants of matrix metalloproteinases with abdominal aortic aneurysm: a systematic review and meta-analysis.Heart. 2014; 100: 295-302
- An update on the hyper-IgE syndromes.Arthritis Res. Ther. 2012; 14: 228
Article Info
Publication History
Published online: January 19, 2017
Accepted:
January 18,
2017
Received in revised form:
December 31,
2016
Received:
October 28,
2016
Identification
Copyright
Published by Elsevier Ireland Ltd.
