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Abstract Details

Vasculitis in the Central Nervous System: Treatment and Outcomes in a Large Single-Center Cohort
Autoimmune Neurology
P9 - Poster Session 9 (5:30 PM-6:30 PM)
6-014

CNS vasculitis is inflammation of blood vessel walls in the brain or spine that can be life-threatening and/or result in ischemic strokes. Markers suggestive of CNS inflammation include cerebrospinal fluid (CSF) pleocytosis and/or elevated protein level, and on neuroaxis imaging, the presence of parenchymal, leptomeningeal, or vessel wall enhancement. At the time of symptom onset, the diagnostic evaluation focuses on classifying the size of the involved vessels and determining if the vasculitis is a primary angiitis of the CNS or if it is occurring secondary to another process elsewhere in the body, such as a systemic vasculitis, connective tissue disorder, infection, malignancy, or toxic/drug exposure.

To describe the demographics, treatment, and outcomes of patients presenting with vasculitis involving the central nervous system (CNS) at a major referral center covering a five-state catchment area in the Intermountain West.

A retrospective chart review for patients within the University of Utah Health system queried all International Classification of Diseases (ICD) 9 codes for 437.4 (Cerebral arteritis) and ICD10 codes for I67.7 (Cerebral arteritis, not elsewhere classified) with one encounter with a neurologist. Of the 144 total patients identified between January 2012 to October 2022, we further confirmed the diagnosis based on neurologists’ review of each record and categorized patients further to determine if the CNS presentation of vasculitis was primary, secondary, or indeterminate/uncertain.

We described the demographic characteristics of vasculitis patients with CNS involvement seen at the University of Utah, as well as presenting symptoms, relevant comorbidities, distinct imaging findings (including a focus on imaging modality), CSF abnormalities, treatments, and outcomes. 

This is a comprehensive characterization of patients with vasculitis involving the CNS in a large area of the Intermountain West. Understanding the differences in these patient characteristics, symptoms, and variability in diagnostic and treatment plans will inform better, more efficient approaches. 

Authors/Disclosures
Yoji Hoshina, MD (University of Utah Health)
PRESENTER
Dr. Hoshina has nothing to disclose.
Alen Delic Alen Delic has nothing to disclose.
Ka-Ho Wong (U of U Neurology Clinic) The institution of Mr. Wong has received research support from The Sumaira Foundation .
Stephanie Lyden, MD Dr. Lyden has nothing to disclose.
Robert Kadish, MD Dr. Kadish has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. The institution of Dr. Kadish has received research support from Alexion Pharmaceuticals.
Stacey Clardy, MD, PhD, FAAN (University of Utah) Dr. Clardy has received personal compensation for serving as an employee of Veterans Health Administration (VHA). Dr. Clardy has received personal compensation for serving as an employee of University of Utah Health. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for AstraZeneca/Alexion. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amgen/Horizon. Dr. Clardy has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Neurology/AAN Publications. The institution of Dr. Clardy has received research support from Sumaira Foundation for NMO. The institution of Dr. Clardy has received research support from NIH/NINDS. The institution of Dr. Clardy has received research support from SRNA. The institution of Dr. Clardy has received research support from Alexion/AstraZeneca. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving as a AAN Summer Meeting CoDirector Travel and Lodging with AAN. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving as a Grand Rounds Travel/Lodging/Honoraria with U of Iowa. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving as a Grand Rounds Travel/Lodging/Honoraria with Barrow Neurological Institute. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving as a Grand Rounds Travel/Lodging/Honoraria with Beaumont Health. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving as a Grand Rounds Travel/Lodging/Honoraria with Stanford. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving as a Grand Rounds Travel/Lodging/Honoraria with Cleveland Clinic.