Sally El Sammak1, Max Herman2, Anudeep Nakirikanti3, Gabriela Bou4, Zachery Rohm5, Siddharama Pawate5, William Tyor6, Diana Vargas7, Spencer Hutto8
1Emory University, 2Emory School of Medicine, 3Emory University School of Medicine, 4Emory University Department of Neurology, 5Vanderbilt University Medical Center, 6Atlanta VAMC, 7Emory Healthcare, 8Emory University: Neurology Residency Program
Objective:
To retrospectively explore outcomes of neurosarcoidosis treated with rituximab
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
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