{
  "abstract": "Introduction/Purpose Reversible cerebral vasoconstriction syndrome(RCVS) is commonly encountered. Clinical scales, such as RCVS2, and non-invasive cerebrovascular imaging are used to differentiate it from primary CNS angiitis and secondary vasospasm. Patients with inflammatory disorders may have higher exposure to vasoactive triggers and other factors that can precipitate RCVS, which may lead to more complicated clinical courses.Materials and Methods We present a patient with a complicated course 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 and treatment heterogeneity for this population.Results A 51-year-old female with systemic lupus erythematosus vs undifferentiated connective tissue disorder on mycophenolate mofetil and hydroxychloroquine, presented with worst headache of life accompanied with blurry vision, vomiting and unsteady gait. She experienced two episodes of thunderclap headache the week prior. 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. Examination revealed mild right upper extremity drift. She had supratherapeutic INR (4.58). CT head demonstrated right parafalcine SAH with small bilateral frontoparietal intraparenchymal hemorrhages. CT angiography showed multifocal distal arterial narrowing without aneurysm. CSF and blood analyses were noninflammatory. The RCVS2 score was 10, and a diagnosis of RCVS was favored. Despite normalizing the INR, discontinuation of potential offending agents such as MMF, fluoxetine, and hydroxychloroquine, and treatment with oral nimodipine, she developed a new left hemiparesis with worsening vasoconstriction on repeat imaging. 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. Systematic review of the literature identified 16 prior cases ( table 1). Rheumatologic conditions included SLE, APLA, lupus nephritis, and others. Presentations included infarcts, PRES, and hemorrhage. Most patients had triggers including steroids, 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.Conclusion RCVS is a clinicoradiological syndrome of vasoconstriction, and complicated courses have been described with comorbid rheumatologic disease. While the mainstay treatment of RCVS is removal of offending agents and oral calcium channel blockers, we propose that angiography and intra-arterial vasodilator therapy can offer dual diagnostic as well as therapeutic benefits in severe or refractory cases.Disclosures F. Kanakhara: None. M. Nahhas: None. R. Regenhardt: None. S. Sheth: None.Abstract E-292 Table 1",
  "authors": [
    {
      "affiliations": [
        "UTHealth Houston, Houston, TX"
      ],
      "name": "F Kanakhara"
    },
    {
      "affiliations": [
        "UTHealth Houston, Houston, TX"
      ],
      "name": "M Nahhas"
    },
    {
      "affiliations": [
        "UTHealth Houston, Houston, TX"
      ],
      "name": "R Regenhardt"
    },
    {
      "affiliations": [
        "UTHealth Houston, Houston, TX"
      ],
      "name": "S Sheth"
    }
  ],
  "title": "E-292 Diagnosis and management of reversible cerebral vasoconstriction syndrome with a systemic rheumatological disorder: a case and updated review of the literature",
  "uid": "ea7ff08a-3f0a-5796-8a5a-8382212d8f99"
}