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Neurosarcoidosis: Longitudinal experience in a single-center, academic healthcare system
Jennifer Lord1, M Mateo Paz Soldan1, Jonathan Galli1
1From the Department of Neurology (J.L., M.M.P.S., J. Galli, R.B., L.D.D., J. Klein, J.R., J. Greenlee, S.L.C.), University of Utah; George E. Wahlen Veterans Affairs Medical Center (J.L., M.M.P.S., J. Galli, S.L.C.); Department of Radiology and Imaging Sciences (K.L.S.), and Departments of Internal Medicine and Bioinformatics (J. Kresser), University of Utah, Salt Lake City.
Objective:
To characterize patients with neurosarcoidosis within the University of Utah healthcare system, including demographics, clinical characteristics, treatment, and long-term outcomes.
Methods:
We describe the clinical features and outcomes of patients with neurosarcoidosis within the University of Utah healthcare system (a large referral center for 10% of the continental United States by land mass). Patients were selected who met the following criteria: (1) at least one International Classification of Diseases Clinical Modification, 9th revision code 135 or International Classification of Diseases Clinical Modification, 10th revision code D86* (sarcoidosis) and (2) at least one outpatient visit with a University of Utah clinician in the Neurology Department within the University of Utah electronic health record.
Results:
We identified 56 patients meeting the study criteria. Thirty-five patients (63%) were women, and most patients (84%) were white. Twelve patients (22%) met the criteria for definite neurosarcoidosis, 36 patients (64%) were diagnosed with probable neurosarcoidosis, and 8 patients (14%) were diagnosed with possible neurosarcoidosis. A total of 8 medications were used for the treatment of neurosarcoidosis. Prednisone was the first-line treatment in 51 patients (91%). Infliximab was the most effective therapy, with 87% of patients remaining stable or improving on infliximab. Treatment response for methotrexate and azathioprine was mixed, and mycophenolate mofetil and rituximab were the least effective treatments in this cohort.
Conclusions:
This is a comprehensive characterization of neurosarcoidosis within a single healthcare system at the University of Utah that reports long-term response to treatment and outcomes of patients with neurosarcoidosis. Our results suggest the use of infliximab as a first-line therapy for neurosarcoidosis.
