Abstract
Immunoglobulin 4 (IgG4)-related systemic disease (IgG4-RSD) is a systemic inflammato-ry disease characterized by elevation of serum IgG4. IgG4-RSD can affect any organ in the body, and the list of organs associated with this condition is growing steadily. IgG4-related cardiovascular dis-ease affects the coronary arteries, heart valves, myocardium, pericardium, aorta, pulmonary and pe-ripheral vessels. Echocardiography is the most commonly used non-invasive imaging method. Com-puted tomography angiography (CTA) can assess aortitis, periarteritis and coronary aneurysms. Cor-onary CTA is fast, offers high spatial resolution and a wide coverage field of view. Cardiac magnetic resonance imaging (CMR) offers a comprehensive evaluation of the cardiovascular system including cardiac function, extent of myocardial fibrosis, characterise cardiac masses with different pulse se-quences and guide to further treatment. Fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT) can provide important information about the extent of disease, the presence of active inflammation and the optimum biopsy site. In general, the role of diagnostic imag-ing includes establishing the diagnosis, detecting complications, guiding biopsy and documenting re-sponse to therapy.
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