Abstract Details

Title Rituximab in CNS Sarcoidosis: A Multi-center Retrospective Series

Topic Autoimmune Neurology

Presentation(s) S38 - Autoimmune Neurology: Peripheral Autoimmunity, Paraneoplastic Disease, Checkpoint Inhibitors, and Neurosarcoidosis (5:18 PM-5:30 PM)

Poster/Presentation
Number 010

Background Highly effective immunotherapies for neurosarcoidosis remain limited largely to tumor necrosis factor alpha inhibitors, but rituximab, a monoclonal antibody that depletes B cells by targeting CD20, has been suggested as an agent to consider in refractory cases. Its effectiveness, however, is largely unexplored

Objective To retrospectively explore outcomes of neurosarcoidosis treated with rituximab

Design/Methods Patients from two US medical centers were included if sarcoidosis was pathologically confirmed and rituximab was used to treat CNS manifestations of the disease. Those with less than 12 months of follow-up were excluded. Once beyond 8 weeks of treatment, failure of rituximab was defined as a similar inflammatory burden on MRI with persistent symptoms (lack of effect), worsening inflammatory burden on MRI with relevant symptoms (relapse or disease progression), or inability to taper prednisone to less than 10 mg per day at six months after initial infusion (failure to spare steroids).

Results

Fourteen patients with neurosarcoidosis were included. Diagnosis was confirmed by non-neurologic biopsies in 9/14 (64.3%) and neurologic biopsies in 5/14 (35.7%). At rituximab initiation, mean age was 47.6 years (+/- 12.53), duration of neurosarcoidosis was 32.5 months (+/- 33.25), mRS was 2 (+/- 0.96), and number of preceding attacks was 2 (+/- 1.5). Rituximab, alone 5/14 (35.7%) or in combination with other immunosuppressants (9/14, 64.3%), was used first or second line in 9/14 (64.3%). Failure occurred in 10/14 (71.4%): relapse or disease progression in 9/14 (64.3%) and failure to spare steroids in 1/14 (7.1%). Mean time to first relapse was 14 months (+/- 14.7). Rituximab was used as third line or later in 3/4 responsive patients. Mean clinical and radiographic follow-up durations post-rituximab were 52.9 months (+/- 36.4) and 43.2 months (+/- 33.1), respectively.

Conclusions Though a majority of patients failed rituximab, a small subset of patients may respond, including the treatment-refractory

Authors/Disclosures
Sally El Sammak, MD (Emory University) PRESENTER	Dr. El Sammak has nothing to disclose.
Max Herman, MD (Emory School of Medicine)	Dr. Herman has nothing to disclose.
Anudeep S. Nakirikanti, MD	Mr. Nakirikanti has nothing to disclose.
Gabriela A. Bou, MD (Emory University School of Medicine)	Dr. Bou has nothing to disclose.
Zachery Rohm, MD	Dr. Rohm has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for EMD Serono.
Siddharama Pawate, MD (Vanderbilt University Medical Center)	The institution of Dr. Pawate has received research support from Genentech. The institution of Dr. Pawate has received research support from Biogen.
William R. Tyor, MD, FAAN (Atlanta VAMC)	The institution of Dr. Tyor has received research support from NIH. The institution of Dr. Tyor has received research support from VHA. The institution of Dr. Tyor has received research support from NIH. The institution of Dr. Tyor has received research support from VHA. The institution of Dr. Tyor has received research support from NIH.
Diana Vargas, MD (Emory Healthcare)	Dr. Vargas has nothing to disclose.
Spencer Hutto, MD (Emory University: Neurology Residency Program)	Dr. Hutto has nothing to disclose.