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. 2019 Oct-Dec;22(4):389-394.
doi: 10.4103/aian.AIAN_365_19. Epub 2019 Oct 25.

Plasma Exchange as a First Line Therapy in Acute Attacks of Neuromyelitis Optica Spectrum Disorders

B L Kumawat  1 , Reenu Choudhary  1 , C M Sharma  1 , Deepak Jain  1 , Ashwini Hiremath  1
Affiliations

Plasma Exchange as a First Line Therapy in Acute Attacks of Neuromyelitis Optica Spectrum Disorders

B L Kumawat et al. Ann Indian Acad Neurol. 2019 Oct-Dec.

Abstract

Background and aims: Neuromyelitis optica spectrum disorder (NMOSD) is a demyelinating disorder of central nervous system with deleterious effects. At present Intravenous corticosteroids are used for the relapse as the first line of treatment, but with only a class evidence III-IV. Having an underlying humoral immune mechanism in the pathogenesis of NMOSD and as it is rightly said that "Time is Cord and Eyes", delaying the time to start plasma exchange (PLEX) awaiting favorable outcome in response to corticosteroids is detrimental for the patient. Hence, PLEX may be a promising first line therapeutic approach in the management of severe attacks of NMOSD. The aim of this study is to evaluate the efficacy of PLEX as the first line of treatment for the acute attacks in patients with NMOSD that is being largely used as an add-on therapy for more than 10 years, and also to define the time opportunity window for the starting of PLEX.

Methods: The study analysed the therapeutic efficacy and safety profile of PLEX as a first line therapy in 30 patients diagnosed with NMOSD over a period of 30 months. PLEX was performed using a Hemonetics Mobile Collection System plus machine with due written consent including the risks and benefits of the treatment that is being proposed to the patient/relative in their own language.

Results: A total of 30 patients were analysed, out of which 16 were females and rest males. 85% of the patients were in the age group of 25-35 years. All the patients had severe Expanded Disability Status Scale (EDSS) scores at the baseline, and 73.33% showed significant improvement following PLEX. The only predictor of good outcome was the time to PLEX i.e shorter delay betters the outcome.

Conclusion: The study ascertained the importance of early PLEX as a therapeutic intervention in severe attacks of NMOSD irrespective of their Anti-Aquaporin 4 (AQP4) antibody status.

Keywords: first line therapy; Corticosteroids; NMOSD; PLEX; time window.

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Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1
Figure 1
(a) This figure illustrates the sites of expression of aquaporin-4 (AQP4) in the central nervous system (CNS). AQP4 is expressed on astrocyte ‘foot-like’ processes at the blood-brain barrier. (b) AQP4-Abs (IgG) are produced systemically by mature B-cells, and upon crossing the blood-brain barrier, activate complement-mediated astrocyte damage. There is relative preservation of myelin initially. The inflammatory milieu consists of neutrophils and eosinophils. Reprinted with permission from Whittam D, Wilson M, Hamid S, et al. What's new in neuromyelitisoptica? A short review for the clinical neurologist. J Neurol 2017;264:2330-44[15]
Figure 2
Figure 2
Mean EDSS score at different time points
Figure 3
Figure 3
Correlation between time to PLEX and EDSS score Percentage Improvement
See this image and copyright information in PMC

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