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

Abstract

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.

Diagnostic Value of Vessel Wall Imaging to Determine the Timing of Extracranial‒Intracranial Bypass for Moyamoya Syndrome Associated with Active Sjögren's Syndrome: A Case Report

“…resulting in neurological sequelae. 5 Neither MR-VWI nor EC-IC bypass was not performed in that case. On the other hand, a case with bilateral MCA, posterior cerebral artery, and basilar artery stenosis, reported in Japan, showed complete resolution of the luminal steno-occlusive change on MR angiography after the immunosuppressant use, 4 regardless of MR-VWI findings.…”

“…However, the treatment outcome varied, although one report did not describe the outcome. 9 Two of four pharmacologically managed patients showed favorable outcomes without repetitive ischemic stroke events, [4][5][6][7] whereas, both patients who underwent bypass surgery in addition to pharmacological management showed favorable outcomes. 3,8 Based on the current literature, we therefore considered that EC-IC bypass is the current treatment of choice for major cerebral artery involvement associated with Sjögren's syndrome, particularly in the Asian population.…”

“…A growing number of reports have showen that primary Sjögren's syndrome, the 2 nd most common chronic autoimmune disorder in Western countries, is characterized by lymphocytic infiltration of exocrine glands and other organs, 1 and rarely involves the major cerebral arteries, leading to ischemic stroke. [3][4][5][6][7][8][9] The seven previous case reports for this rare condition were all published from Asian countries, as shown in the Table 1. For life-threatening illness in Sjögren's syndrome, including the progressive major cerebral artery occlusion causing ischemic stroke, cyclophosphamide is needed.…”

“…1,2 In rare cases (only seven cases reported in the literature) it involves the major cerebral arteries, leading to ischemic stroke. [3][4][5][6][7][8][9] While the exact mechanism underlying this rare condition is unclear, previous case reports primarily addressed this extraglandular manifestation by systemic therapy, including steroidal, non-steroidal antiinflammatory, and antiplatelet agents, with varied clinical outcomes. Systemic anti-inflammatory therapy led to reversible stenosis of the large cerebral arteries in one case, 4 while in another case, it resulted in an irreversible stenosis leading to recurrent acute ischemic stroke with neurological sequelae.…”

“…Systemic anti-inflammatory therapy led to reversible stenosis of the large cerebral arteries in one case, 4 while in another case, it resulted in an irreversible stenosis leading to recurrent acute ischemic stroke with neurological sequelae. 5 There has also been a report showing the efficacy of extracranial-intracranial (EC-IC) bypass in combination with the anti-inflammatory therapy, even in an active inflammatory state. 8 However, the optimal timing for the EC-IC bypass in this rare condition is yet to be determined.…”