Reversible cerebral vasoconstriction syndrome(RCVS) mimics primary CNS angiitis. Patients with inflammatory disorders have higher exposure to vasoactive triggers and other factors that can precipitate RCVS, which may lead to more complicated clinical courses. 

We present a complicated case of RCVS with notable history of a rheumatological disorder. Further, we systematically review the literature for RCVS cases with comorbid rheumatological diagnoses to understand the clinical course, triggers and treatment heterogeneity.

A 51-year-old female with SLE vs undifferentiated connective tissue disorder on mycophenolate mofetil and hydroxychloroquine, presented with recurrent thunderclap headaches and focal deficits. She also had a history of mechanical aortic valve requiring aspirin and warfarin, implantable cardioverter-defibrillator, hypertension, depression on fluoxetine, and chronic pain on cannabidiol. CT head demonstrated SAH. CT angiography showed multifocal distal arterial narrowing. CSF and blood analyses were noninflammatory, but MR black-blood imaging was inconclusive. Despite discontinuation of potential offending agents and treatment with oral nimodipine, she deteriorated. Subsequent digital subtraction angiography confirmed the diffuse distal vasoconstriction. Verapamil 10mg was administered in each ICA resulting in angiographic improvement. She had resolution of weakness and returned to her functional baseline within 2 weeks. 

Our systematic review identified 15 prior cases, associated with rheumatologic conditions included SLE, APLA, systemic sclerosis and others. RCVS-PACNS overlap syndrome was also mentioned. Presentations included infarcts, PRES, and hemorrhage. Most patients had triggers including steroids(n=9), immunosuppressants and vasoactive agents. Patients often got serial imaging and increasing doses of calcium channel blockers for variable durations, alongside removal of triggers. However, IA vasodilator therapy was used infrequently.