Case report reveals cryptogenic stroke from progressive internal carotid artery occlusion due to IgG4-RD, suggesting increased recognition of vascular involvement in the disease.
Immunoglobulin G4–related disease (IgG4-RD) is a systemic fibroinflammatory disorder capable of multiorgan involvement, though its association with cerebral vasculitis and large-vessel occlusion remains rare and poorly characterized. We report a novel case of cryptogenic ischemic stroke secondary to progressive internal carotid artery (ICA) stenosis and occlusion, ultimately attributed to IgG4-RD. The patient exhibited multifocal hypermetabolic lesions on positron emission tomography–computed tomography, correlating with arterial wall thickening and subsequent pathologic confirmation of IgG4-positive plasma cell infiltration. This case highlights IgG4-RD as an underrecognized cause of progressive ICA stenosis and occlusion, offering critical insights into the diagnostic work-up of cryptogenic stroke and expanding the spectrum of vascular manifestations in IgG4-RD.
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Bu et al. (2026) studied this question.
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