Abstract Details

Title First-line Induction Therapy for Hospitalized Patients with Primary CNS Vasculitis

Topic Autoimmune Neurology

Presentation(s) P3 - Poster Session 3 (5:00 PM-6:00 PM)

Poster/Presentation
Number 2-011

Objective NA

Background Rituximab has been used as an alternative first-line induction therapy to cyclophosphamide for primary central nervous system vasculitis (PCNSV). This study characterized clinical characteristics and induction therapy outcomes in a hospitalized cohort with PCNSV.

Design/Methods This was a single-center retrospective case-control study. Kruskal-Wallis test was used to compare median mRS improvement to worst mRS across groups. Intergroup comparisons and post-hoc covariate analysis were done using the Wilcoxon Rank-Sum test.

Results 47 patients were treated with cyclophosphamide (n= 34, 72%), rituximab (n=8, 17%), or combined induction therapy (n= 5, 11%). Mean age was 50. 51% of the cohort was female, 57% White, 19% Black, and 8.5% Hispanic. 39/46=85% had multifocal lesions; 13/39=33% had abnormal MRA brain; 18/28=64% abnormal catheter angiogram; 2/10=20% abnormal vessel wall MRI. Brain biopsy confirmed vasculitis in 27/35=77%. Only 60% (26/43) had CSF pleocytosis, and 17% of patients without pleocytosis still had biopsy-proven vasculitis. Cyclophosphamide led to improved median mRS change compared to rituximab at 6 months (p = 0.015), but not at 12 months (p=0.4201). However, when including only biopsy-confirmed vasculitis cases, there was no difference in induction therapy (p = 0.447). Treatment with cyclophosphamide >3g (n=26) led to a greater change in median mRS compared to <3g (n=7) (-1.5 vs -1.0). Initiating treatment within 1 month of symptom onset led to better mRS outcomes at 6 months (p= 0.024) and 12 months (p=0.044). Age less than 50, Black/Hispanic race/ethnicity, necrotizing pathology or ICU admission did not impact mRS outcomes. No rituximab patients experienced CTCAE greater than 3, whereas 13% of cyclophosphamide patients had grade 4 CTCAE.

Conclusions Cyclophosphamide treatment led to improved 6-month mRS in PCNSV compared to rituximab, though this was not significant when including only biopsy-confirmed cases. Initiating treatment <1 month from symptom onset may lead to improved outcomes. Multicenter prospective randomized controlled trials are warranted.

Authors/Disclosures
Edith Graham, MD (Northwestern University) PRESENTER	Dr. Graham has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Novartis. Dr. Graham has received personal compensation in the range of $500-$4,999 for serving as a Consultant for TG Therapeutics. The institution of Dr. Graham has received research support from F. Hoffman-La Roche Ltd. The institution of Dr. Graham has received research support from Novartis.
Rebecca DiBiase, MD (University of California San Francisco)	Dr. DiBiase has nothing to disclose.
Sangri Kim, MD (Northwestern Memorial Hospital)	Dr. Kim has nothing to disclose.
Revika Singh	Miss Singh has nothing to disclose.
Maxwell E. Shramuk, MS	Mr. Shramuk has nothing to disclose.
Anisha Dua, MD	Dr. Dua has received personal compensation in the range of $500-$4,999 for serving as a Consultant for novartis. Dr. Dua has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Amgen. Dr. Dua has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sanofi. Dr. Dua has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for abbvie.
Karan S. Dixit, MD (Northwestern University)	Dr. Dixit has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Servier.
Roumen D. Balabanov, MD (Northwestern University)	Dr. Balabanov has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Dr. Balabanov has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Balabanov has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Balabanov has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Biogen. The institution of Dr. Balabanov has received research support from NextCure. The institution of Dr. Balabanov has received research support from Biogen. The institution of Dr. Balabanov has received research support from NINDS.

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