We report on an unusual case presenting with a cardiac pseudo-tumour on echocardiogram, which corresponded to a large soft tissue mural thickening around the mid-right coronary artery. There were similar but not as thick mural lesions around other parts of the coronary arteries. The so-called “pigs-in-a-blanket” sign on computed tomography (CT) scan was pathognomonic of IgG4 coronary arteritis. The IgG4 level was grossly elevated at more than 10 times the upper limit of normal. Positron emission tomography (PET)-CT scans with 18F-fluoro-deoxy-glucose (FDG) and 68Ga-Fibroblast Activation Protein Inhibitor (FAPI) were performed to assess the extent of organ involvement of the IgG4-related disease. The patient was treated with 8 injections of rituximab with good serological response. However, the coronary arteritis findings on CT scan remained unchanged.
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