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Abstract Details

Recurrent Stroke in Giant Cell Arteritis Despite Immunotherapy
Cerebrovascular Disease and Interventional Neurology
P4 - Poster Session 4 (5:30 PM-6:30 PM)
3-028
To describe the rare but important cerebrovascular complications of Giant Cell Arteritis (GCA) and to report an unfortunate relapse when immunosuppression was switched from cyclophosphamide to tocilizumab.

Stroke is a rare but important complication of GCA that occurs in 3-4% of patients and is typically due to stenosis of carotid and/or vertebral or basilar arteries. Despite aggressive steroid and/or immunosuppressive therapy, there is high morbidity and mortality in this patient population.

Case report and literature review.

We report a 59-year-old male who initially presented with vasculitis of the lower extremities. While on steroids, he developed strokes in multiple vascular territories.  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 a local rheumatologist .  Two months later, the patient had progression of vessel stenosis and suffered additional strokes.  Imaging revealed expanding infarctions and extensive hypoperfusion.  Despite interventions to augment cerebral perfusion, the patient passed away.

This case highlights several important features of this rare complication of GCA: the predilection for the dural entry point of cerebral blood vessels, the progression of the disease despite steroids, and the low threshold clinicians should have to escalate treatment in these cases.  To our knowledge, this is the first patient with CNS involvement of GCA who was treated with tocilizumab. While this medication has been recently validated as a treatment of GCA, it has not been studied in patients with CNS involvement. The CNS penetration of Tocilizumab is poor and the relapse of our patient following the switch from cyclophosphamide to tocilizumab is suspicious.  The clinician should use caution in using this medication in patients with GCA of the CNS until further evidence supports its use.

Authors/Disclosures
Benjamin C. Cox, MD (University of Alabama at Birmingham)
PRESENTER
The institution of Dr. Cox has received research support from American Epilepsy Society. The institution of Dr. Cox has received research support from Brain and Behavior Research Foundation.
Jimmy R. Fulgham, MD (Mayo Clinic) No disclosure on file
James P. Klaas, MD, FAAN An immediate family member of Dr. Klaas has received publishing royalties from a publication relating to health care.