A case of reversible cerebral vasoconstriction syndrome associated with anti-phospholipid antibody syndrome and systemic lupus erythematosus

The pathomechanisms and treatment strategy for rare presentations of reversible cerebral vasoconstriction syndrome (RCVS) with anti-phospholipid syndrome (APS) remain to be determined. We report a 67-year-old woman with APS who presented with ischemic stroke due to RCVS. She was treated with low-dose cilostazol and lomerizine hydrochloride, which resulted in functional improvement and recovery of vasoconstriction within 12 weeks. Her plasma endothelin-1 level was decreased after relief of vasoconstriction, compared with the pre-treatment condition. Increased plasma endothelin-1 may be related to the underlying pathomechanism of RCVS with APS, against which cilostazol and lomerizine hydrochloride could be effective.
Highlights • We described a case of reversible cerebral vasoconstriction syndrome (RCVS) with anti-phospholipid syndrome (APS). • Treatment with low-dose cilostazol and lomerizine hydrochloride could therapeutic for RCVS with APS. • These treatments may alleviate the endothelin-1-related cerebral vascular dysfunction in RCVS/APS.