Immunoglobulin 4 (IgG4)-related systemic disease (IgG4-RSD) is a systemic inflammatory
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
disease affects the coronary arteries, heart valves, myocardium, pericardium, aorta, pulmonary
and peripheral vessels. Echocardiography is the most commonly used non-invasive imaging
method. Computed tomography angiography (CTA) can assess aortitis, periarteritis and coronary
aneurysms. Coronary 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 sequences 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 imaging includes establishing the diagnosis, detecting complications, guiding
biopsy and documenting response to therapy.