Rituximab in pathologically confirmed sarcoidosis affecting the central nervous system: a multi-center retrospective study

Abstract

Background: Rituximab, a monoclonal antibody that depletes B cells by targeting CD20, has been suggested as a treatment option for neurosarcoidosis, but evidence to support its use is limited.

Methods: Patients from four US medical centers with CNS sarcoidosis treated with rituximab were retrospectively studied. After 8 weeks of treatment, rituximab failure was defined as a similar inflammatory burden on MRI with persistent symptoms, worsening inflammatory burden on MRI with relevant symptoms, or inability to taper prednisone to less than 10 mg daily 6 months after initial infusion.

Results: Nineteen patients were included. At rituximab initiation, duration of neurosarcoidosis was 27.0 months (± 32.1), mRS was 2.0 (± 1.0), number of preceding attacks was 2 (± 1.8), and treatments previously tried were 1.0 (± 1 0.7). Patients received a median 4 g (± 2.8) over 19.0 months (± 16.8). Rituximab, alone (8/19, 42.1%) or in combination with other immunosuppressants (11/19, 57.9%), was used first or second line in 12/19 (63.2%). Failure occurred in 14/19 (73.7%): relapse or disease progression in 12/14 (85.7%) and failure to spare steroids in 2/14 (14.3%). Median time to first relapse was 7.0 months (± 13.5), occurring at a median 4.0 months (± 2.6) since the last dose. Among the five responsive patients, rituximab was used as third line or later in 4/5 (80.0%), and most (4/5, 80.0%) exhibited multiple cranial neuropathies.

Conclusions: While rituximab failed in most patients with neurosarcoidosis, a small subset may benefit, particularly those with multiple cranial neuropathies, even after failure of other medications.

Keywords: B-cell depletion; Disease-modifying therapy; Neurosarcoidosis; Rituximab; Sarcoidosis.