Department of Neurology, University of Missouri, Columbia, Missouri, USA.
Division of Nephrology, Department of Internal Medicine, University of Missouri, Columbia, Missouri, USA.
J Clin Apher. 2021 Oct;36(5):727-736. doi: 10.1002/jca.21923. Epub 2021 Jul 9.
Despite the availability of several immunomodulatory therapies, about 20% of myasthenia gravis (MG) patients remain refractory to conventional treatments. There is limited evidence to support the use of maintenance therapeutic plasma exchange (TPE) therapy for refractory generalized MG.
Retrospective chart review of 14 patients with refractory generalized MG treated for 12 months with maintenance TPE therapy. Outcome measures were myasthenia gravis composite (MGC) score, myasthenia gravis activities of daily living (MG-ADL), number of acute exacerbations, medication changes, and adverse events. Data were collected at 3 monthly intervals for 12 months before and after initiation of TPE therapy.
Clinically meaningful reductions in mean MG-ADL (>2 points) (mean MG-ADL score: 9.9 ± 0.5; 12-month pre-TPE to 5.2 ± 0.9; 12-month post-TPE) and MGC (>3 points) (mean MGC score: 25.2 ± 1.6; 12-month pre-TPE to 11.7 ± 1.4; 12-month post-TPE) were observed at 3 months following initiation of TPE and were maintained up to 12 months in all patients. After 12 months of TPE therapy, all patients had a significant reduction in daily prednisone and pyridostigmine use. Patients previously on IVIG or rituximab therapy were successfully weaned off both treatments. There was a significant reduction in acute MG exacerbations; 7.8 ± 1.1 mean exacerbations/patient (12-month pre-TPE) to 2 ± 1.1 mean exacerbations/patient (12-month post-TPE).
Over a period of 12 months, maintenance TPE therapy improved MG-ADL, and MGC with decreased immunosuppressant requirement, while being well-tolerated.
尽管有几种免疫调节疗法可供选择，但约 20%的重症肌无力（MG）患者对常规治疗仍无反应。对于难治性全身性 MG，维持性治疗性血浆置换（TPE）治疗的证据有限。
对 14 例难治性全身性 MG 患者进行回顾性图表分析，这些患者接受了 12 个月的维持性 TPE 治疗。主要观察指标为重症肌无力综合评分（MGC）、重症肌无力日常生活活动评分（MG-ADL）、急性加重次数、药物改变和不良反应。在开始 TPE 治疗前和治疗后 3 个月收集数据，共收集 12 个月。
在开始 TPE 治疗 3 个月后，MG-ADL（≥2 分）（MG-ADL 评分：9.9±0.5；12 个月前 TPE 至 5.2±0.9；12 个月后 TPE）和 MGC（≥3 分）（MGC 评分：25.2±1.6；12 个月前 TPE 至 11.7±1.4；12 个月后 TPE）的平均评分均有临床意义的降低，并且在所有患者中均持续至 12 个月。在 TPE 治疗 12 个月后，所有患者的泼尼松和吡啶斯的明的日剂量均显著减少。以前接受 IVIG 或利妥昔单抗治疗的患者成功停用了这两种治疗药物。MG 急性加重次数显著减少，从平均 12 个月前 TPE 的 7.8±1.1 次/患者减少到 12 个月后 TPE 的 2±1.1 次/患者。
在 12 个月的时间里，维持性 TPE 治疗改善了 MG-ADL 和 MGC，减少了免疫抑制剂的需求，同时耐受性良好。
