The association of recurrent stroke and immunoglobulin G4-related arteritis in the central nervous system: a case report

BACKGROUND: Immunoglobulin G4-related disease (IgG4-RD) is a systemic immune-mediated fibroinflammatory disease. Histopathology is the key to diagnosis. Although aortitis/periaortitis is a phenotype of IgG4-related disease, the relationship between cerebrovascular disease and IgG4-related disease remains unclear. METHODS: We report a 60-year-old man with recurrent stroke induced by IgG4-related arteritis and present the pathological findings of the patient's superficial temporal artery and middle cerebral artery. RESULTS: Immunohistochemistry demonstrated scattered plasma cells in the intima and adventitia of the superficial temporal artery, with a small number of IgG4-positive cells observed (10-15 cells per high-power field). CONCLUSIONS: Case reports and studies examining the association between IgG4-related neurological arteritis and stroke are limited. Although a definitive link between IgG4-related arteritis (IgG4-RA) and stroke has not been established, IgG4-related arteritis should be considered as an etiology in patients with recurrent idiopathic stroke.