Disease course and treatment of neurosarcoidosis in a single center cohort study of 113 cases
Affan M, Kananeh M, Schultz L, Elias S, and Cerghet M. Disease course and treatment of neurosarcoidosis in a single center cohort study of 113 cases. Neurology 2017; 88(16 Suppl 1).
Objective: To describe treatment and outcomes in a cohort of patients with neurosarcoidosis. Background: Neurosarcoidosis (NS) is estimated to be in present in up to 25% of patients with systemic sarcoidosis. Current treatment of NS, including steroids, immunosuppressants, relies on case reports as there are no controlled trials. Design/Methods: The study setting was a large, integrated health care system serving southeastern Michigan. Electronic medical records for 113 patients who met the criteria for NS (Zajicek, 1999) were reviewed. Data on medication usage and outcomes were analyzed by observing clinical response, imaging and modified Rankin score (mRS). Good response was defined as improvement or no progression in disease activity. Poor response was defined as worsening of symptoms, imaging or relapses. Chi-squared tests were used to compare the outcomes for most recent MRI. Nonparametric tests were used to test the associations. All testing was done at the 0.05 level. SAS version 9.4 was used for data analyses. Results: Of 113 patients, 58% were female and 73% African American. The average duration of follow up was 7.6 years. 61.5% had a monophasic disease, 31.5% had relapses and 11.5% died. Of the 113 patients, 107 (94%) received glucocorticosteroids. Additional immunosuppressant agents were used in 72%. Good response was noted with TNFa (59%), methotrexate (52%), azathioprine (32%). Poor response was noted with mycophenolate mofetil (44%), hydroxycholoroquine (24%). Out of 65 patients with MRI outcomes, 11% had resolution, 8% improved, 35% remained stable. mRS remained stable in 65% but worsen in in 13%. Abstract Conclusions: We present the largest case series of NS treatment and outcomes. Most patients had response to glucocorticosteroids. TNFa and methotrexate provided the highest good outcomes when combination therapy was used. Treatment remains challenging and prospective studies as well as clinical trials are needed to improve outcome in NS.
16 (Suppl 1)