Acute Ischemic Stroke as Initial Presentation of Primary Sjögren’s Syndrome: Case Report

Medical literature has documented rare instances where an acute ischemic stroke serves as the first clinical manifestation of primary Sjögren’s syndrome (PSS). While Sjögren’s syndrome is typically characterized by dryness of the eyes and mouth, neurological complications can occur, sometimes presenting as the primary symptom before other systemic signs are identified.

Recent case reports, including a study published in Cureus, highlight the challenge of diagnosing this autoimmune condition when it presents as a stroke. These cases illustrate that ischemic stroke as a presenting manifestation of primary Sjögren’s syndrome is considered extremely rare.

Neurological Manifestations and Vasculitis

Neurological complications in Sjögren’s syndrome are often linked to vasculitis. In one reported case, a 30-year-old female experienced an ischemic stroke as the initial manifestation of vasculitis associated with the syndrome. Radiological investigations in that instance showed an infarct in the right middle cerebral artery (MCA), and laboratory findings revealed a positive ANA profile.

Another case report detailed a presentation of large-vessel vasculitis and cerebral infarction as the initial manifestation of the syndrome. This suggests that the inflammation of blood vessels can lead to a lack of blood flow to the brain, resulting in a stroke.

Clinical Presentations of Recurrent Stroke

In some instances, the condition may present not as a single event but as recurrent strokes. A report published in Neurohospitalist described a 50-year-old male who presented for evaluation after experiencing two episodes of discrete focal neurological deficits over a six-week period.

Neuro-imaging for this patient revealed evidence of acute-subacute bihemispheric infarcts. This case further underscores the rarity of ischemic stroke acting as the primary presenting symptom of primary Sjögren’s syndrome.

Understanding Primary Sjögren’s Syndrome

Primary Sjögren’s syndrome is a systemic autoimmune disease. While it is most commonly known for affecting the exocrine glands—leading to sicca symptoms like dry eyes and dry mouth—it can have extraglandular manifestations that affect multiple organ systems, including the central nervous system.

When the disease affects the vascular system, it can cause vasculitis, which is the inflammation of the blood vessel walls. This inflammation can restrict blood flow or cause vessel occlusion, leading to cerebral infarctions (strokes).

The identification of these cases is critical for clinicians because the absence of typical sicca symptoms may lead to a delay in diagnosing the underlying autoimmune cause of a stroke. Identifying a positive ANA profile or other markers of autoimmunity can help steer the diagnostic process toward Sjögren’s syndrome in patients who do not fit the traditional profile of stroke risk factors.

Diagnostic Challenges

The primary challenge in these cases is the atypical nature of the presentation. Because ischemic strokes are more commonly associated with cardiovascular risk factors such as hypertension or diabetes, an autoimmune etiology may not be the first consideration for medical teams.

• Radiological imaging, such as MRI, is used to identify the location and extent of the infarct, such as the middle cerebral artery.
• Laboratory tests, including ANA profiles, help identify the systemic autoimmune nature of the condition.
• Clinical history is used to identify recurrent neurological deficits that may suggest a systemic vascular issue rather than a localized cardiovascular event.