Abstract

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.

Keywords: IgG4-RSD, cardiovascular complications, coronary aneurysm, aortitis, computed tomography angiography, CMR.