Neurosarcoidosis: Longitudinal experience in a single-center, academic healthcare system

Neurol Neuroimmunol Neuroinflamm. 2020 May 13;7(4):e743. doi: 10.1212/NXI.0000000000000743. Print 2020 Jul.

Abstract

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.

MeSH terms

  • Academic Medical Centers / statistics & numerical data
  • Adult
  • Antirheumatic Agents / therapeutic use*
  • Central Nervous System Diseases / diagnosis
  • Central Nervous System Diseases / drug therapy*
  • Central Nervous System Diseases / epidemiology*
  • Central Nervous System Diseases / ethnology
  • Female
  • Glucocorticoids / therapeutic use*
  • Health Services / statistics & numerical data
  • Humans
  • Immunologic Factors / therapeutic use*
  • Infliximab / therapeutic use*
  • Longitudinal Studies
  • Male
  • Middle Aged
  • Sarcoidosis / diagnosis
  • Sarcoidosis / drug therapy*
  • Sarcoidosis / epidemiology*
  • Sarcoidosis / ethnology
  • Utah / epidemiology

Substances

  • Antirheumatic Agents
  • Glucocorticoids
  • Immunologic Factors
  • Infliximab

Supplementary concepts

  • Neurosarcoidosis