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Sjögren’s syndrome (SS) is a multi-systemic autoimmune disease. Central nervous system (CNS) involvement is rare. We report a case of large-vessel vasculitis and cerebral infarction as an initial presentation of SS. Neurological complications in SS is often due to peripheral neuropathy through small-vessel vasculitis. In rare cases, CNS involvement can occur, including acute ischaemic strokes (AIS), linked to both accelerated artherosclerosis and large-vessel vasculitis. Management of SS-related AIS remains complex due to the scarcity of evidence, although a role for immunomodulation and biologics remains promising. Clinicians should remain vigilant in identifying SS as a cause for stroke, especially in the young.

Recurrent acute ischaemic strokes as the primary presentation of Sjögren’s syndrome