Plasma exchange versus intravenous immunoglobulin in AChR subtype myasthenic crisis: A prospective cohort study

Yuan Wang; Xiao Huan; Kexin Jiao; Qilong Jiang; Li-Ying Goh; Jianquan Shi; Zhiguo Lv; Jianying Xi; Jie Song; Chong Yan; Jie Lin; Wenhua Zhu; Xinfang Zhu; Zhirui Zhou; Rong Xia; Sushan Luo; Chongbo Zhao; Pan-Yangtze River Delta Alliance for Neuromuscular Disorders (PYDAN)

doi:10.1016/j.clim.2022.109058

Plasma exchange versus intravenous immunoglobulin in AChR subtype myasthenic crisis: A prospective cohort study

Clin Immunol. 2022 Aug:241:109058. doi: 10.1016/j.clim.2022.109058. Epub 2022 Jun 9.

Authors

Yuan Wang¹, Xiao Huan², Kexin Jiao², Qilong Jiang³, Li-Ying Goh⁴, Jianquan Shi⁵, Zhiguo Lv⁶, Jianying Xi², Jie Song², Chong Yan², Jie Lin², Wenhua Zhu², Xinfang Zhu¹, Zhirui Zhou⁷, Rong Xia⁸, Sushan Luo⁹, Chongbo Zhao¹⁰; Pan-Yangtze River Delta Alliance for Neuromuscular Disorders (PYDAN)

Affiliations

¹ Department of Blood Transfusion, Huashan Hospital Fudan University, Shanghai 200040, China.
² Huashan Rare disease centre, Department of Neurology, Huashan Hospital Fudan University, Shanghai 200040, China; National Center for Neurological Disorders, Shanghai 200040, China.
³ First Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangzhou, China.
⁴ Shanghai Medical College, Fudan University, Shanghai 200032, China.
⁵ Department of Neurology, Nanjing First Hospital, Nanjing Medical University, Nanjing, Jiangsu, China.
⁶ Changchun University of Chinese Medicine Department of Neurology, The Affiliated Hospital of Changchun University of Chinese Medicine, Changchun 130000, China.
⁷ Radiation Oncology Center, Huashan Hospital, Fudan University, Shanghai, China.
⁸ Department of Blood Transfusion, Huashan Hospital Fudan University, Shanghai 200040, China. Electronic address: xiarong@huashan.org.cn.
⁹ Huashan Rare disease centre, Department of Neurology, Huashan Hospital Fudan University, Shanghai 200040, China; National Center for Neurological Disorders, Shanghai 200040, China. Electronic address: luosushan@fudan.edu.cn.
¹⁰ Huashan Rare disease centre, Department of Neurology, Huashan Hospital Fudan University, Shanghai 200040, China; National Center for Neurological Disorders, Shanghai 200040, China. Electronic address: zhao_chongbo@fudan.edu.cn.

PMID: 35690385
DOI: 10.1016/j.clim.2022.109058

Abstract

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.

Keywords: Acetylcholine receptors; Intravenous immunoglobulin; Myasthenic crisis; Plasma exchange.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Autoantibodies
Humans
Immunoglobulins, Intravenous* / therapeutic use
Myasthenia Gravis*
Plasma Exchange
Prospective Studies
Receptors, Cholinergic

Substances

Autoantibodies
Immunoglobulins, Intravenous
Receptors, Cholinergic