Abstract

OBJECTIVE To conduct systematic review and meta-analysis for the efficacy of therapeutic plasma exchange (TPE) for neuromyelitis optica spectrum disorder (NMOSD) with an acute attack. METHODS Systematic review was performed using EMBASE and OVID/Medline database. The eligible studies must be the studies of NMOSD patients treated with TPE during the acute phase. They must report treatment outcomes using either Expanded Disability Status Scale (EDSS) or visual acuity (VA) before and after the therapy. Pooled mean difference (MD) was then calculated by combining MDs of each study using the random-effects model. RESULTS Fifteen studies were identified; eleven with 241 NMOSD patients reported EDSS outcome and four studies with 103 NMOSD reported visual outcomes. The meta-analysis demonstrated a significantly decreased in EDSS after TPE treatment for NMOSD with an acute attack with the pooled MD of 0.83 (95% CI, 0.26-1.40; I(2) 69%) comparing pretreatment to immediate posttreatment and 2.13 (95% CI, 1.55-2.70; I(2) 31%) comparing pretreatment to posttreatment at 6 months to 1-year follow-up. Unfortunately, only one of the four studies evaluating visual outcomes reported standard deviation in association with mean LogMAR; therefore, the meta-analysis cannot be conducted. Nonetheless, all studies consistently demonstrated the benefit of TPE with improved VA and/or LogMAR after treatment. INTERPRETATION This systematic review and meta-analysis showed the benefit of TPE during the NMOSD attack with a significantly improved disability status immediately after treatment and during follow-up.

Abstract

BACKGROUND Therapeutic plasma exchanges (TPE) has been recommended for neuromyelitis optica spectrum disorders (NMOSD) as a rescue therapy after nonresponding from the high-dose steroid and as an early therapy in severe attacks. We performed a systematic review to evaluate whether therapeutic plasma exchange (TPE) is better than conventional intravenous methylprednisolone (IVMP) in neuromyelitis optica spectrum disorders (NMOSD) patients. METHODS Systematic search was conducted in five databases: PubMed, Embase, Scopus, Web of Science, and CENTRAL for randomized controlled trials and observational studies of TPE compared to intravenous steroid in NMOSD patients with neurological or visual outcomes in English without publication date restriction. Quality assessment was performed using ROB2 and ROBINS-I. The meta-analysis was done using a random-effects model. Pooled risk ratio (RR) or mean difference with a 95% CIs of efficacy outcomes included the Expanded Disability Status Scale (EDSS), visual acuity, and LogMAR were measured. RESULTS Of 3439 potential studies, seven were included in the systematic review (1211 attacks; 433 patients) and three studies were included in the meta-analysis. Compared to high dose steroid alone, the add-on TPE increases a chance for the returning of EDSS to baseline at discharge (RR 3.02, 95% CI 1.34-6.81) and last follow-up (RR 1.68, 95% CI 1.01-2.79) as well as improves visual acuity at last follow-up. CONCLUSION TPE as an add-on therapy to high-dose steroid injection during an acute attack in NMOSD patients is associated with returning to baseline EDSS at discharge and last follow-up, and a trend to have a lower disability at 6-12 months.

Abstract

BACKGROUND Our previous retrospective study demonstrated that NMOSD patients with an acute attack who did not respond to IVMP alone, however, showed further significant improvement after treatment with PLEX at 6 month-follow-up. OBJECTIVE To compare the efficacy between treatments with intravenous methylprednisolone (IVMP) with subsequent add-on plasma exchange (PLEX) and a combination of simultaneous IVMP and PLEX in neuromyelitis optica spectrum disorders (NMOSD) patients with a severe acute attack. METHOD We conducted a prospective, randomized, controlled, pilot study of the treatments for a severe acute attack in NMOSD patients. RESULTS There were eleven AQP4-positive NMOSD patients in the study. One received only IVMP, five received IVMP with subsequent add-on PLEX treatment, and the other five received simultaneous IVMP and PLEX treatment. The attacks comprised myelitis (57.1%) and optic neuritis (42.9%). Both treatments with IVMP followed by subsequent add-on PLEX when needed (not-respond to IVMP treatment) and a combination treatment of simultaneous IVMP+PLEX from the outset showed clinical improvement measured by EDSS at 6 months follow-up, compared to those at the attacks (p-value=0.07 in IVMP add-on PLEX group and p-value=0.05 in IVMP+PLEX group), respectively. Although, a trend of a better outcome stratified by EDSS toward early PLEX initiation with IVMP+PLEX treatment was observed at 6 months follow-up, however not significantly. CONCLUSION Early treatment with PLEX should be encouraged especially in NMOSD with a severe acute attack.