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An Immunohistopathologic Study to Profile the Folate Receptor Beta Macrophage and Vascular Immune Microenvironment in Giant Cell Arteritis
Published on: February 8, 2019
Recurrent Stroke in Giant Cell Arteritis Despite Immunotherapy
Benjamin C Cox1, Jimmy R Fulgham, James P Klaas
1Department of Neurology, Mayo Clinic, Rochester, MN.
Insights
Giant cell arteritis (GCA) can cause rare but severe strokes, even with steroid treatment. Aggressive immunosuppression is crucial for managing these dangerous cerebrovascular complications.
Area of Science:
- Neurology
- Rheumatology
- Vascular Medicine
Background:
- Giant cell arteritis (GCA) is a systemic vasculitis primarily affecting medium and large arteries.
- Cerebrovascular complications, though rare, represent a significant cause of morbidity and mortality in GCA patients.
Observation:
- A 59-year-old male with GCA presented with lower extremity vasculitis, followed by multiple strokes despite corticosteroid therapy.
- Angiography revealed stenosis at the dural entry points of bilateral carotid and vertebral arteries.
- Disease progression and additional strokes occurred even after initiating cyclophosphamide and later switching to tocilizumab.
Findings:
- GCA can manifest with cerebrovascular events, particularly affecting vessels at the dura mater's entry point.
- Corticosteroids alone may be insufficient to prevent stroke progression in some GCA cases.
- Aggressive immunosuppressive therapy escalation is necessary, but outcomes remain poor in severe cases.
Implications:
- This case underscores the importance of recognizing and promptly treating cerebrovascular complications in GCA.
- Early escalation of immunosuppressive therapy may be critical for improving outcomes.
- The predilection for dural entry vessels highlights a specific vulnerability in GCA-related stroke.
Objective:
To describe rare but important cerebrovascular complications of giant cell arteritis (GCA).
Case Report:
We report a 59-year-old man who initially presented with vasculitis of the lower extremities. While on steroids, he developed strokes in multiple vascular territories. The conventional angiogram showed stenosis of bilateral carotid and vertebral vessels as they entered the dura. Temporal artery biopsy confirmed GCA. He began cyclophosphamide treatment, which stabilized his clinical course; however, this was switched to tocilizumab by an outside rheumatologist. Two months later, the patient had progression of vessel stenosis and suffered additional strokes. Despite interventions to augment cerebral perfusion, the infarctions continued to expand and the patient passed away.
Conclusions:
This case highlights several important features of strokes in GCA: the predilection for the dural entry point of cerebral blood vessels, the progression of disease despite steroids, and the need to quickly escalate treatment in these cases. As seen in our patient, however, this disease carries high morbidity and mortality and patients often have poor outcomes despite aggressive immunosuppression.
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