Tamer Ghanayem1, Sage Hewitt1, Siddharama Pawate1, James Eaton1, Francesca Bagnato1, Shailee Shah1
1Vanderbilt University Medical Center
Objective:
The purpose of this study is to demonstrate the potential utility of infliximab in possible neurosarcoidosis.
Background:
Sarcoidosis is a multisystem granulomatous inflammatory disorder with nervous system involvement in 5-15% of cases. Diagnosis and treatment is challenging in the 17% of patients with isolated neurosarcoidosis. If neural biopsy is unobtainable, these patients can only be diagnosed as “possible neurosarcoidosis.” Infliximab, a tumor necrosis factor (TNF) inhibitor, is efficacious in the management of neurosarcoidosis but it remains unclear whether it would be beneficial in possible neurosarcoid patients.
Design/Methods:
This is a retrospective single-center case series of four glucocorticoid-refractory possible neurosarcoidosis patients meeting 2018 Neurosarcoidosis Diagnostic Criteria after exclusion of neoplastic and infectious mimics.
Results:
Four male patients, median age 42.7 years (range 36.3-57.8), presented with inflammatory CSF (median 61 cells/mm3[range 8-403]) and imaging abnormalities (nodular optic perineuritis and meningomyelitis) compatible with neurosarcoidosis without systemic manifestations. All patients received high-dose intravenous/prolonged-oral steroids (median prednisone duration 4.5 months [range 3.1-7.7]) before infliximab initiation. Due to disease progression, infliximab was initiated in all patients for median 7.4 months (range 2.9-31.1) with concomitant methotrexate (n=2), and mycophenolate (n=1) to mitigate anti-drug antibody formation risk. Prednisone was tapered off in two patients and the remaining patients are on 5 and 20 milligrams of prednisone with ongoing tapers. There was near complete resolution of MRI contrast enhancement in all patients over a median span of 5.7 months (range 3.1-32.7) and a reduction in mean EDSS and mRS by 2.3 and 1.3 since treatment onset, respectively. Overall, the median duration of follow-up was 1.1 years (0.5-2.6).
Conclusions:
Infliximab may prove to be beneficial in patients with possible neurosarcoidosis and minimize steroid exposure and toxicity. However, particular care should be given to excluding infectious, especially mycobacterial and fungal, and neoplastic causes before exposing patients to TNF inhibitors.