Short delay to initiate plasma exchange is the strongest predictor of outcome in severe attacks of NMO spectrum disorders
Short delay to initiate plasma exchange is the strongest predictor of outcome in severe attacks of NMO spectrum disorders
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DOI：
10.1136/jnnp-2017-316286
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发表时间：
2018-04-01
影响因子：
11
通讯作者：
Cabre, Philippe
中科院分区：
文献类型：
--
作者：
Bonnan, Mickael;Valentino, Rudy;Cabre, Philippe
介绍严重发作的视神经肌病谱系障碍（NMO-SD）通过血浆置换（PLEX）作为一种连续治疗得到改善。最初的研究未能证明PLEX治疗的延迟影响临床结局;然而，PLEX总是在较晚的时候使用。我们研究延迟PLEX启动严重的视神经炎和脊髓攻击的NMO-SD.Methods我们所有的患者遭受攻击的NMO-SD，在我们的中心治疗PLEX的临床后果，回顾性地考虑列入。主要结局定义为完全改善。次要不良/良好结局分别定义为Delta扩展残疾状态量表（EDSS）的高/低三分之一（晚期减基线EDSS）。结果60例患者中，符合NMO-SD标准（2015）的患者占92%。纳入115例发作，并在临床发作后中位数7天（0-54）内接受PLEX治疗。恢复完全改善的概率从第0天给予PLEX的50%持续下降至第20天后的1%-5%。通过多变量分析，基线损害和PLEX延迟与完全改善的概率相关（OR 5.3; 95%CI 1.8 - 15.9）。减少PLEX延迟也影响了良好的次要结局，但不差的次要outcome.Conclusions这些结果证实了改善的临床效益早期启动PLEX严重攻击NMO-SD。仅在类固醇失败后将PLEX视为补救治疗可能有害。
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.