Effects of immunotherapies and clinical outcomes in neurosarcoidosis: a retrospective cohort study

Abstract

Introduction

Neurosarcoidosis is associated with a significant degree of morbidity and mortality and its treatments are varied and complex. There is a paucity of information in current literature on patterns of treatment and long term outcomes. This study aimed to evaluate the clinical outcomes and responses to immunosuppressive therapy in a large cohort of neurosarcoidosis patients .

Methods

We enrolled 80 patients with a diagnosis of neurosarcoidosis. Prescription patterns and clinical outcomes before and after treatment and differences between the treatment groups were compared using Kruskal–Wallis and Mann–Witney U tests.

Results

Patients with cranial mononeuropathy other than optic neuropathy were more likely to be treated with steroids alone whereas patients with other presentations were likely to require second and third level treatments. These included azathioprine, methotrexate, mycophenolate, infliximab, and cyclophosphamide often used in combination. Prednisolone alone at onset failed in 67% of patients but appeared most effective in those with isolated facial nerve palsy. Patients treated with prednisolone plus a standard immunosuppression first line generally did well except for those with brain parenchymal disease and /or hydrocephalus who responded better to the addition of infliximab, or cyclophosphamide. Triple therapy with prednisolone + azathioprine + infliximab was associated with significantly greater improvement on the Modified Rankin Scale than prednisolone alone whether used first line (p = 0.001 corrected) or subsequently (p = 0.021 corrected). Overall favourable outcomes in the form of improvement of MRS were reported in 87%,

Conclusions

Our results provides evidence that early immunosuppressive treatments, with azathioprine, methotrexate and infliximab could effectively improve clinical outcomes in many patients with neurosarcoidosis.

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Affiliations

Authors

Contributions

TA: Tarunya Arun has received support for scientific meetings and speaker fees from Sanofi Genzyme, Merck Serono and Novartis. JP: Jacqueline Palace is partly funded by highly specialised services to run a national congenital myasthenia service and a neuromyelitis service. She has received support for scientific meetings and honorariums for advisory work from Merck Serono, Biogen Idec, Novartis, Teva, Chugai Pharma and Bayer Schering, Alexion, Roche, Genzyme, MedImmune, EuroImmun, MedDay, Abide and ARGENX, and grants from Merck Serono, Novartis, Biogen Idec, Teva, Abide, VielaBio and Bayer Schering. She has received grants from the MS society, Guthie Jackson Foundation, NIHR, Oxford Health Services Research Committee, EDEN, MRC, GMSI, and John Fell for research studies.

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Correspondence to Tarunya Arun.

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Arun, T., Palace, J. Effects of immunotherapies and clinical outcomes in neurosarcoidosis: a retrospective cohort study. J Neurol (2021). https://doi.org/10.1007/s00415-021-10421-z

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Keywords

  • Neurosarcoidosis (146)
  • Immunosuppression
  • Infliximab
  • Outcomes
  • Modified rankin scale