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A Patient With Multiple Coronary Artery Aneurysms

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A man in his early 40s was admitted to the hospital because of acute chest pain. Laboratory test results were notable for high serum concentrations of IgG4 (5650 mg/dL; to convert to grams per liter, multiply by 0.01) and C-reactive protein (78.1 mg/L; to convert to nanomoles per liter, multiply by 9.524). Coronary computed tomographic angiography was performed (Figure, A and B). It revealed a large aneurysm (90 mm × 48 mm) with a thrombosis in the left anterior descending artery (LAD) and a perivascular soft-tissue mass involving the patent proximal right coronary artery (RCA) and left circumflex artery, which showed homogeneous moderate enhancement on contrast-enhanced images. Coronary computed tomographic angiography with cinematic rendering (Figure, C) showed multiple mass lesions around the RCA and LAD. Invasive coronary angiography results were unable to visualize the LAD but did identify a hypervascular mass in the proximal RCA.

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IgG4-associated coronary artery aneurysms

D. Coronary artery bypass graft surgery

A unique immune-mediated fibroinflammatory disorder, IgG4-related disease is characterized by elevated serum IgG4 levels and tumefied lesions that may concurrently or consecutively affect a wide variety of organs.13 It is most common in middle-aged or elderly men. Diagnosis of IgG4-related disease is based on histological findings that show IgG4-positive plasma cell and lymphocytic infiltration, fibrosis with storiform features, and obliterative phlebitis. Elevated serum IgG4 levels also indicate IgG4-related disease.

Cases of IgG4-associated coronary artery involvement have rarely been reported. They can result in ischemic heart disease, as in this case, or sudden cardiac death. Because IgG4-associated coronary inflammation occurs in the adventitia, it can result in inflammatory pseudotumor, perivascular fibrosclerotic thickening, luminal stenosis, and aneurysmal dilatation. The typical mistletoe sign in coronary computed tomographic angiography is indicative of the diagnosis of IgG4-associated coronary artery disease,4 as this case shows. Positron emission tomography and computed tomography are also useful in evaluation and follow-up of IgG4-related disease with coronary artery involvement; these can show hypermetabolic coronary artery masses and identify alternative biopsy sites.5

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Article Information

Corresponding Author: Long Jiang Zhang, MD, PhD, Department of Medical Imaging, Jinling Hospital, Medical School of Nanjing University, 305 Zhongshan East Rd, Xuanwu District, Nanjing, Jiangsu Province 210002, China (kevinzhlj@163.com).

Published Online: December 19, 2018. doi:10.1001/jamacardio.2018.4268

Conflict of Interest Disclosures: None reported.

References
1.
Yong  YR, Lath  N, Cheah  FK, Ng  YL.  Pictorial essay: uncommon causes of coronary artery encasement.  J Cardiovasc Comput Tomogr. 2016;10(5):424-429. doi:10.1016/j.jcct.2016.07.004PubMedGoogle ScholarCrossref
2.
Mavrogeni  S, Markousis-Mavrogenis  G, Kolovou  G.  IgG4-related cardiovascular disease: the emerging role of cardiovascular imaging.  Eur J Radiol. 2017;86:169-175. doi:10.1016/j.ejrad.2016.11.012PubMedGoogle ScholarCrossref
3.
Barbu  M, Lindström  U, Nordborg  C, Martinsson  A, Dworeck  C, Jeppsson  A.  Sclerosing aortic and coronary arteritis due to IgG4-related disease.  Ann Thorac Surg. 2017;103(6):e487-e489. doi:10.1016/j.athoracsur.2016.12.048PubMedGoogle ScholarCrossref
4.
Maurovich-Horvat  P, Suhai  FI, Czimbalmos  C,  et al.  Coronary artery manifestation of Ormond disease: the “mistletoe sign”.  Radiology. 2017;282(2):356-360. doi:10.1148/radiol.2016160644PubMedGoogle ScholarCrossref
5.
Huang  HL, Fong  W, Peh  WM, Niraj  KA, Lam  WW.  The utility of FDG PET/CT in IgG4-related disease with a focus on coronary artery involvement.  Nucl Med Mol Imaging. 2018;52(1):53-61. doi:10.1007/s13139-017-0494-5PubMedGoogle ScholarCrossref
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