IgG4-related Cardiovascular Disease from the Aorta to the Coronary Arteries: Multidetector CT and PET/CT

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.Abbreviations: CVD = cardiovascular disease, ECG = electrocardiography, FDG = fluorine 18 fluorodeoxyglucose, IgG4 = immunoglobulin G4, RCA = right coronary artery, SUV max = maximum standardized uptake value, TBR = target-to-background ratio

The utility of PET/CT in large vessel vasculitis

“…These generally result from perivascular tumefactive lesions 30 . In addition, PET/CT can distinguish cardiovascular features of IgG4-related disease including aortitis, periaortitis, arteritis, periarteritis, atherosclerosis, and pericarditis 31 . Moreover, areas of aortic inflammation may be apparent on PET/CT scans in patients with PsO and RA, even after adjustment for cardiovascular risk factors 32 .…”

Sudden Cardiac Death due to Coronary Artery Vasculitis in a Patient with Relapsing Polychondritis

“…Arteritis, periarteritis, and aneurysmal dilation of the coronary arteries have all been described with relation to IgG4-related cardiac disease. When the coronary arteries are involved, patients may be asymptomatic or minimally symptomatic but remain at risk for myocardial ischemia and sudden cardiac death [18].…”

It's Not All in Your Head: Thoracic Manifestations of Neurologic Diseases and Disorders

“…Pleural manifestations of IgG4-RD include nodules/thickening and effusion (4). Cardiovascular involvement in IgG4-RD includes aortitis-periaortitis ( Fig 1B), large>medium vessel arteritis-periarteritis, and inflammatory aneurysms (6). Yabusaki et al found that the most common site of IgG4-related vasculitis was the iliac arteries (35%), followed by the infrarenal aorta (33%), thoracic aorta and first branches of the thoracic aorta (8%), suprarenal abdominal aorta (6%), and first branches of the abdominal aorta (5%) (7).…”

“…Yabusaki et al found that the most common site of IgG4-related vasculitis was the iliac arteries (35%), followed by the infrarenal aorta (33%), thoracic aorta and first branches of the thoracic aorta (8%), suprarenal abdominal aorta (6%), and first branches of the abdominal aorta (5%) (7). The coronary arteries (arteritis and aneurysms) and pericardium can also be involved (6).…”