Can an eye stroke be caused by an autoimmune disease?

Autoimmune Diseases Can Cause Eye Stroke

Yes, eye stroke (central retinal artery occlusion or CRAO) can be caused by autoimmune diseases, particularly autoimmune vasculitis conditions like giant cell arteritis. 1 While most cases of eye stroke result from embolic occlusion, the arteritic subtype caused by inflammatory processes represents an important autoimmune etiology.

Autoimmune Causes of Eye Stroke

Giant Cell Arteritis (GCA)

• Most common autoimmune cause of arteritic CRAO 1
• Affects medium and large extracranial arteries, including distal branches of carotid artery
• Results in occlusive intimal hyperplasia
• Can cause permanent vision loss in 8.2% of patients with biopsy-proven GCA 1
  • 1.6% specifically due to CRAO
  • 0.4% due to cilioretinal artery occlusion
• May present with concomitant arteritic anterior ischemic optic neuropathy (AION) and choroidal ischemia 1

Other Autoimmune Vasculitides

• Primary CNS angiitis/vasculitis can cause stroke (2.4 cases per 1 million patient-years) 1
• Systemic lupus erythematosus, mixed connective tissue disease, and Takayasu arteritis can cause multiple cerebral infarcts 2
• Antiphospholipid syndrome with lupus anticoagulant can lead to ischemic stroke 3

Mechanisms of Autoimmune-Related Eye Stroke

1. Direct inflammatory vessel damage - Vasculitis affecting the central retinal artery 1
2. Hypercoagulable states - Autoimmune conditions can create prothrombotic environments 2
3. Embolic phenomena - Detected on transcranial Doppler in autoimmune-related strokes 2
4. Occlusive vasculopathy - Inflammatory processes causing vessel narrowing 4

Clinical Presentation of Eye Stroke

• Sudden, painless monocular loss of visual acuity and peripheral vision 1
• Initial visual acuity is "count fingers" or worse in >80% of patients 1
• Ipsilateral relative afferent pupillary defect 1
• Funduscopic findings include:
  • Retinal edema (whitening)
  • Cherry red spot
  • Attenuated arteries 1

Diagnostic Approach for Suspected Autoimmune Eye Stroke

1. Urgent ophthalmologic evaluation - Funduscopic examination to confirm CRAO
2. Laboratory testing:
   • Erythrocyte sedimentation rate (ESR)
   • C-reactive protein (CRP) - CRP >10 mg/L associated with increased VTE risk 1
   • Complete blood count
   • Autoimmune markers (ANA, RF, ANCA)
3. Imaging:
   • Carotid/cerebral vessel imaging
   • High-resolution MRI to evaluate for vasculitis 2
4. Consider temporal artery ultrasound - May show halo sign in GCA 1
5. Temporal artery biopsy if GCA suspected - Should not delay treatment 1

Treatment of Autoimmune-Related Eye Stroke

For Giant Cell Arteritis:

• Immediate initiation of high-dose glucocorticoids within 24 hours of symptom onset 1
  • Critical to reduce risk of permanent blindness and increase chance of visual recovery
  • Do not wait for biopsy confirmation if GCA is strongly suspected
• Consider adjunctive therapy with:
  • Methotrexate or tocilizumab - Reasonable to lower recurrent stroke risk 1
  • Avoid infliximab - Associated with recurrent ocular symptoms and disease activity 1

For Primary CNS Angiitis:

• Induction therapy with glucocorticoids and/or immunosuppressants 1
• Long-term maintenance with steroid-sparing immunosuppressants:
  • Options include cyclophosphamide, mycophenolate mofetil, azathioprine, methotrexate, or rituximab 1
  • Choice should be based on individual agent profile and comorbidities

Important Caveats and Pitfalls

1. Don't miss GCA - Delay in treatment can lead to permanent blindness; maintain high clinical suspicion in patients >50 years
2. Don't wait for biopsy - Start steroids immediately if GCA is suspected
3. Consider multiple mechanisms - Autoimmune-related strokes often involve multiple pathophysiological processes 2
4. Watch for bilateral involvement - Some patients may develop contralateral AION or bilateral CRAO 1
5. Monitor for steroid complications - Long-term steroid use requires careful monitoring and bone protection

Early recognition and prompt treatment of autoimmune-related eye stroke are essential to prevent permanent vision loss and reduce the risk of recurrent events.