血浆置换与静脉注射免疫球蛋白治疗乙酰胆碱受体亚型肌无力危象的前瞻性队列研究。
Plasma exchange versus intravenous immunoglobulin in AChR subtype myasthenic crisis: A prospective cohort study.
机构信息
Department of Blood Transfusion, Huashan Hospital Fudan University, Shanghai 200040, China.
Huashan Rare disease centre, Department of Neurology, Huashan Hospital Fudan University, Shanghai 200040, China; National Center for Neurological Disorders, Shanghai 200040, China.
出版信息
Clin Immunol. 2022 Aug;241:109058. doi: 10.1016/j.clim.2022.109058. Epub 2022 Jun 9.
Myasthenic crisis (MC) is a life-threatening state with respiratory failure in patients with myasthenia gravis (MG). The fast-acting immunomodulatory therapies for treating MC included plasma exchange (PE) and intravenous immunoglobulin (IVIG). However, the efficacy and the impact on antibody changes remained unknown. We prospectively followed 40 anti-acetylcholine receptors (AChR) antibody-positive MC patients who received either PE (n = 12) or IVIG (n = 28) at crisis. PE was associated with a reduced ICU stay length (p = 0.018) and an early response by the average changes in MGFA-QMG (p = 0.003), MMT (p = 0.020), and ADL (p = 0.011) at one-week off-ventilation. However, the clinical efficacy was equally comparable in both groups after 1 month. Post-treatment hemoglobin drop was significant in both groups, while IVIG was associated with a significant reduction in anti-AChR antibody titers (p < 0.001). This analysis provides real-world evidence in supporting the use of PE as a fast-acting therapy for shortening the ICU stay in AChR-associated MC.
肌无力危象（MC）是一种危及生命的状态，可导致重症肌无力（MG）患者发生呼吸衰竭。用于治疗 MC 的快速起效的免疫调节疗法包括血浆置换（PE）和静脉注射免疫球蛋白（IVIG）。然而，其疗效和对抗体变化的影响尚不清楚。我们前瞻性地随访了 40 名抗乙酰胆碱受体（AChR）抗体阳性的 MC 患者，他们在危象时分别接受了 PE（n=12）或 IVIG（n=28）治疗。PE 与 ICU 住院时间缩短相关（p=0.018），并且在通气停止一周时，MGFA-QMG（p=0.003）、MMT（p=0.020）和 ADL（p=0.011）的平均变化更早出现反应。然而，在 1 个月后，两组的临床疗效相当。两组在治疗后血红蛋白均明显下降，而 IVIG 与 AChR 抗体滴度显著降低相关（p<0.001）。本分析提供了真实世界的证据，支持使用 PE 作为一种快速起效的疗法，缩短 AChR 相关 MC 的 ICU 住院时间。
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