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Coronary Artery Abnormalities in Hyper-IgE Syndrome

  1. Author:
    Freeman, A. F.
    Avila, E. M.
    Shaw, P. A.
    Davis, J.
    Hsu, A. P.
    Welch, P.
    Matta, J. R.
    Hadigan, C.
    Pettigrew, R. I.
    Holland, S. M.
    Gharib, A. M.

  2. Author Address

    [Freeman, AF; Davis, J; Hsu, AP; Holland, SM] NIAID, Immunopathogenesis Sect, Lab Clin Infect Dis, Bethesda, MD 20892 USA [Avila, EM] Duke Univ, Sch Med, Clin Res Training Program, NIH,Pfizer, Durham, NC USA [Shaw, PA] NIAID, Biostat Res Branch, NIH, Bethesda, MD 20892 USA [Welch, P] NCI, SAIC Frederick, Frederick, MD 21701 USA [Matta, JR; Pettigrew, RI; Gharib, AM] NIDDK, NIH, Bethesda, MD USA [Hadigan, C] NIAID, Immunoregulat Lab, NIH, Bethesda, MD 20892 USA;Freeman, AF (reprint author), NIAID, Immunopathogenesis Sect, Lab Clin Infect Dis, NIH Bldg 10,Room 11N234,10 Ctr Dr, Bethesda, MD 20892 USA;freemaal@mail.nih.gov
    1. Year: 2011
    2. Date: Jun
  2. Journal: Journal of Clinical Immunology
    1. 31
    2. 3
    3. Pages: 338-345
  4. Type of Article: Article
  5. ISSN: 0271-9142
  1. 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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External Sources

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  2. DOI: 10.1007/s10875-011-9515-9
  3. View record in Web of Science®
  4. WOS: 000292556000006

Library Notes

  1. Fiscal Year: FY2010-2011
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