Cerebral large-vessel vasculitis in sjogren's syndrome
The present, published in the in the Journal of Clinical Neurology, demonstrates vasculitis of cerebral large-vessels in a patient with primary Sjogren's syndrome and provides further insight into the pathogenesis of the CNS involvement in Sjogren's syndrome.

A 50-year-old female without vascular risk factors presented with recurrent episodes of right-hemispheric transient ischemic attacks involving left upper limb paresthesia and weakness, with complete recovery of symptoms after several minutes, and two episodes of righthemispheric minor stroke with recovery after 2 days. The patient also reported dryness of the eyes with no joint or cutaneous symptoms.

An examination revealed no focal neurological deficits. The routine blood parameters, ESR, and findings of a CSF study were normal. The antinuclear antibody profile revealed strong positivity for anti-Sjogren's-syndrome-related antigen A(SSA/Ro), anti-Sjogren's-syndrome-related antigen B (SSB/La), and PM-Scl. Schirmer's test was positive for dry-eye syndrome. Head CT revealed infarcts in the right middle cerebral artery (MCA) and anterior cerebral artery (ACA) deep watershed territory, and in the MCA and posterior cerebral artery cortical watershed territory.

CT angiography revealed stenosis of the right supraclinoid and communicating segments of the intracranial internal carotid artery (ICA). Digital-subtraction angiography showed 80% stenosis of the right supraclinoid ICA with normal distal smaller vessels. High-resolution MR vessel wall imaging (HRVWI) revealed smooth homogeneous concentric wall thickening with enhancement involving the communicating segment of the right ICA, the proximal M1 segment of the MCA, and the A1 segment of the ACA, without any significant positive or negative remodeling, suggestive of vasculitis of the terminal right ICA and its branches.

Large-vessel vasculitis secondary to Sjogren's syndrome was diagnosed. The patient was treated for 5 days with intravenous methylprednisolone followed by oral prednisolone, along with mycophenolate mofetil and hydroxychloroquine.

The patient had not experienced any further cerebral ischemic events at a 3-month follow-up.

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