Short delay to initiate plasma exchange is the strongest predictor of outcome in severe attacks of NMO spectrum disorders

J Neurol Neurosurg Psychiatry. 2018 Apr;89(4):346-351. doi: 10.1136/jnnp-2017-316286. Epub 2017 Oct 13.

Abstract

Introduction: Severe attacks of neuromyelitis optica spectrum disorder (NMO-SD) are improved by plasma exchange (PLEX) given as an adjunctive therapy. Initial studies failed to demonstrate a delay of PLEX treatment influenced clinical outcome; however PLEX was always used late. We examine the clinical consequences of delay in PLEX initiation on severe optic neuritis and spinal cord attacks in NMO-SD.

Methods: All of our patients who suffered attacks of NMO-SD, treated in our centre by PLEX, were retrospectively considered for inclusion. Primary outcome was defined as complete improvement. Secondary poor/good outcomes were respectively defined to be the higher/lower third of Delta-Expanded Disability Status Scale (EDSS) (late minus baseline EDSS). Delays from clinical onset to PLEX initiation were categorised for multivariate analysis.

Results: Of the 60 patients included, NMO-SD criteria (2015) were fulfilled in 92%. One hundred and fifteen attacks were included and received PLEX with a median of 7 days (0-54) after clinical onset. The probability to regain complete improvement continuously decreased from 50% for PLEX given at day 0 to 1%-5% after day 20. Through multivariate analysis, the baseline impairment and PLEX delay were associated with the probability to complete improvement (OR 5.3; 95% CI 1.8 to 15.9). Reducing the PLEX delay also influenced the good secondary outcome but not the poor secondary outcome.

Conclusions: These results confirm an improved clinical benefit of early initiation of PLEX during severe attacks of NMO-SD. Perceiving PLEX as a rescue therapy only after steroid failure could be deleterious.

Keywords: Neuromyelitis optica; optic neuritis; plasma exchange; transverse myelitis.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Aquaporin 4 / immunology
  • Autoantibodies / immunology
  • Combined Modality Therapy
  • Early Medical Intervention
  • Female
  • Glucocorticoids / therapeutic use
  • Humans
  • Male
  • Methylprednisolone / therapeutic use
  • Middle Aged
  • Myelin-Oligodendrocyte Glycoprotein / immunology
  • Neuromyelitis Optica / immunology
  • Neuromyelitis Optica / therapy*
  • Optic Neuritis / immunology
  • Optic Neuritis / therapy
  • Plasma Exchange / methods*
  • Retrospective Studies
  • Severity of Illness Index
  • Time-to-Treatment / statistics & numerical data*
  • Treatment Outcome
  • Young Adult

Substances

  • AQP4 protein, human
  • Aquaporin 4
  • Autoantibodies
  • Glucocorticoids
  • MOG protein, human
  • Myelin-Oligodendrocyte Glycoprotein
  • Methylprednisolone