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Primary Sjögren's Syndrome Presenting as Recurrent Ischemic Strokes
Praveen Kesav1, Syed Irteza Hussain1, Preeti Devnani1
1Department of Neurology, Neurological Institute (NI), Cleveland Clinic Abu Dhabi (CCAD), Abu Dhabi, United Arab Emirates.
The Neurohospitalist
|April 14, 2022
Summary
Primary Sjogren's Syndrome (PSS) rarely presents with ischemic stroke. This case highlights a 50-year-old male with Antiphospholipid Syndrome (APS) associated with PSS, presenting with bihemispheric infarcts.
Area of Science:
- Neurology
- Rheumatology
- Immunology
Background:
- Primary Sjogren's Syndrome (PSS) is a systemic autoimmune disease affecting exocrine glands.
- Neurological complications occur in approximately 20% of PSS patients, with peripheral neuropathy being more common than central nervous system (CNS) involvement.
- Central nervous system manifestations (CNS-SS) include stroke, demyelination, meningitis, encephalitis, ataxia, cognitive impairment, and movement disorders.
Observation:
- A 50-year-old male presented with two episodes of focal neurological deficits over six weeks.
- Neuroimaging confirmed acute-subacute bihemispheric infarcts.
- Extensive serological testing revealed positive antiphospholipid antibodies (aPL), antinuclear antibody (IIF-ANA), anti-SS-A/Ro, and anti-RNP antibodies.
Findings:
- Lip biopsy demonstrated characteristic PSS pathology: periductal/periacinar lymphocytic infiltration, acinar atrophy, and interstitial fibrosis.
- The patient's presentation and serological profile were consistent with Antiphospholipid Syndrome (APS) associated with PSS.
Implications:
- This case underscores the rare but significant association of ischemic stroke as a presenting manifestation of PSS.
- Highlights the importance of comprehensive autoimmune workup in patients with unexplained ischemic events.
- Emphasizes the diagnostic challenge and potential severity of CNS involvement in PSS, particularly when co-occurring with APS.
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