IgG4-related periarteritis of the carotid artery

A 74-year-old woman presented with the swelling of the right upper eyelid. Fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) showed abnormal uptake in the left anterior neck as well as in the swelling right lacrimal gland. The cervical uptake was fusiform-shaped (Figure  1a, arrow) and contiguous to the left carotid artery (Figure  1a, arrowhead). No abnormal uptake was found in the other major arteries. Ultrasonography revealed that the cervical lesion corresponded to a homogeneously thickened wall without luminal stenosis/dilatation (Figure  1b, upper). These ultrasonographic findings were similar to ‘mantle sign’, which is usually seen in inflammatory aortic aneurysm on CT.1 The serum Immunoglobulin G4 (IgG4) level was 255  mg/dl (upper normal range: 108 mg/dl). The needle biopsy of the right lacrimal gland revealed marked proliferation of IgG4-positive plasma cells (IgG4+/IgG+plasma cell>70%); the patient met the criteria for the diagnosis of IgG4-related disease, which deterred from the biopsy of the left carotid lesion due to the risk of complication. After corticosteroid treatment for 2 months, the arterial wall thickening was drastically improved on ultrasonography (Figure  1b, lower) as the serum IgG4 level normalized, which highly suggested the diagnosis of IgG4-related periarteritis of the carotid artery.
Source: QJM - Category: Internal Medicine Source Type: research