IgG4-related Cardiovascular Disease from the Aorta to the Coronary Arteries: Multidetector CT and PET/CT
Noriko Oyama-Manabe1, Satoshi Yabusaki1, Osamu Manabe1
1From the Department of Diagnostic and Interventional Radiology (N.O.M., S.Y., F.K., K.K.) and Department of Surgical Pathology (H.K.O.), Hokkaido University Hospital, N14, W5, Kita-ku, Sapporo, Japan 060-8638; and Department of Nuclear Medicine, Hokkaido University Graduate School of Medicine, Sapporo, Japan (O.M.).
Insights
Immunoglobulin G4 (IgG4)-related disease can impact the cardiovascular system. Noninvasive imaging like CT and PET/CT is crucial for diagnosing IgG4-related cardiovascular disease (CVD) and monitoring treatment.
Area of Science:
- Cardiovascular Imaging
- Immunology
- Radiology
Background:
- Immunoglobulin G4 (IgG4)-related disease is a systemic condition that can affect the cardiovascular system, including coronary arteries and large vessels.
- Cardiovascular involvement in IgG4-related disease can lead to severe outcomes like myocardial ischemia and sudden cardiac death.
- Histopathology is the gold standard for diagnosis, but obtaining vessel wall biopsies is challenging, highlighting the need for noninvasive methods.
Purpose of the Study:
- To review the multidetector CT and PET/CT imaging findings of IgG4-related cardiovascular disease (CVD).
- To emphasize the role of noninvasive imaging in the diagnosis and management of IgG4-related CVD.
- To describe the inflammatory processes, quantification of inflammation, and therapeutic response assessment in IgG4-related CVD.
Main Methods:
- Review of multidetector CT and PET/CT imaging characteristics in IgG4-related CVD.
- Discussion of various manifestations including aortitis, periaortitis, arteritis, periarteritis, coronary artery involvement, and pericarditis.
- Description of techniques for evaluating active inflammation and therapeutic response.
Main Results:
- Multidetector CT is effective for primary diagnosis, defining anatomy, and characterizing coronary pathology.
- Electrocardiographically gated CT offers advantages over catheter angiography for coronary assessment.
- Combined PET/CT can identify inflammatory processes and differentiate IgG4-related CVD from atherosclerosis.
Conclusions:
- Noninvasive imaging, particularly multidetector CT and PET/CT, plays a vital role in diagnosing and managing IgG4-related CVD.
- Familiarity with imaging findings is essential for accurate diagnosis and monitoring treatment response.
- These imaging modalities aid in understanding the extent and activity of the inflammatory process in IgG4-related CVD.
Abstract:
Immunoglobulin G4 (IgG4)-related disease can affect the cardiovascular system, including the coronary arteries and pericardium and especially the walls of large and medium-sized vessels. The presence of coronary involvement is critical, as this condition can cause myocardial ischemia or sudden cardiac death. Although histopathologic examination remains the reference standard for detecting organ involvement and diagnosing IgG4-related disease, obtaining biopsy or surgical specimens from the vessel wall is still challenging. Because patients may be only mildly symptomatic, noninvasive imaging evaluation of IgG4-related cardiovascular disease (CVD) has an essential role in not only the diagnosis but also the management of this condition. Multidetector CT is a useful noninvasive examination for establishing the primary diagnosis and defining anatomic landmarks and their relationships. The spectrum of vessel involvement is vast, with varied manifestations. Radiologists should be familiar with inflammatory vasculitis, aneurysmal change, and pseudotumor formation in all vessels and the distribution of these conditions throughout the body. Electrocardiographically gated CT enables accurate, fast, and noninvasive characterization of coronary pathologic conditions and thus has an important advantage over catheter angiography. Combined PET/CT can depict inflammatory processes and help distinguish IgG4-related CVD from atherosclerosis. Familiarity with the PET/CT and CT findings of inflammatory processes involved in IgG4-related CVD is important for accurate diagnosis and evaluation of therapeutic response during follow-up. The multidetector CT and PET/CT characteristics of IgG4-related CVD, such as aortitis, periaortitis, arteritis, and periarteritis and including coronary artery involvement and pericarditis, are reviewed. In addition, the inflammatory process, quantification of active inflammation, and therapeutic response during follow-up associated with IgG4-related CVD are described. Online DICOM image stacks are available for this article. ©RSNA, 2018.
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