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

Wang, Yuan; Huan, Xiao; Jiao, Kexin; Jiang, Qilong; Goh, Li-Ying; Shi, Jian-Quan; Lv, Zhiguo; Xi, Jianying; Song, Jie; Yan, Chong; Lin, Jie; Zhu, Wenhua; Zhu, Xinfang; Zhou, Zhirui; Xia, Rong; Luo, Sushan; Zhao, Chongbo

doi:10.1016/j.clim.2022.109058

Cited by 11 publications

(10 citation statements)

References 35 publications

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“…Safa et al reported that the combination therapies of PSL plus PE or intravenous immunoglobulin (IVIg) may lead to more favorable outcomes than PSL monotherapy in irAE MG ( 8 ). Furthermore, in idiopathic MG, a prospective study revealed that PE was able to improve the symptoms earlier than IVIg ( 9 ). Moreover, PE was unable to be safely introduced under unstable hemodynamic conditions due to the presence of accompanying myocarditis, as PE should be introduced before the progression of irAE myopathy.…”

Section: Discussionmentioning

confidence: 99%

Pembrolizumab-induced Myopathy with Anti-striated Muscle Antibodies Successfully Treated by Plasma Exchange

Takahashi,

Okabayashi,

Soejima

et al. 2023

Intern. Med.

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A 70-year-old woman with advanced endometrial cancer developed right ptosis and muscle weakness in the right quadriceps after pembrolizumab administration. Serum creatine kinase (CK) levels were elevated, and anti-striated muscle antibodies were positive. On magnetic resonance imaging, the right vastus lateral muscle showed an abnormal signal. She was diagnosed with pembrolizumab-induced myopathy. We initiated plasma exchange (PE), and the ptosis immediately resolved. We then introduced oral corticosteroids, which improved her muscle weakness. We were able to rapidly diagnose her with ocular symptoms and serum CK level elevation. The early initiation of PE might prevent the exacerbation of pembrolizumab-induced myopathy.

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Section: Discussionmentioning

confidence: 99%

Pembrolizumab-induced Myopathy with Anti-striated Muscle Antibodies Successfully Treated by Plasma Exchange

Takahashi,

Okabayashi,

Soejima

et al. 2023

Intern. Med.

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“…Patients with DM1 were enrolled from January 2020 to April 2023 through the collaborative efforts of the Pan-Yangtze River Delta Alliance for Neuromuscular Disorders (PYDAN) network in China [ 8 – 10 ]. Written informed consent to be included in the study was obtained from all patients.…”

Section: Methodsmentioning

confidence: 99%

Clinical features and genetic spectrum of a multicenter Chinese cohort with myotonic dystrophy type 1

Zhong,

Zeng,

et al. 2024

Orphanet J Rare Dis

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Background As the most common subtype of adult muscular dystrophy worldwide, large cohort reports on myotonic dystrophy type I (DM1) in China are still lacking. This study aims to analyze the genetic and clinical characteristics of Chinese Han DM1 patients. Methods Based on the multicenter collaborating effort of the Pan-Yangtze River Delta Alliance for Neuromuscular Disorders, patients with suspected clinical diagnoses of DM1 were genetically confirmed from January 2020 to April 2023. Peak CTG repeats in the DMPK gene were analyzed using triplet repeat-primed PCR (TP-PCR) and flanking PCR. Time-to-event analysis of onset age in females and males was performed. Additionally, detailed clinical features and longitudinal changes from the disease onset in 64 DM1 patients were retrospectively collected and analyzed. The Epworth Sleepiness Scale and Fatigue Severity Scale were used to quantify the severity of daytime sleepiness and fatigue. Results Among the 211 genetically confirmed DM1 patients, the mean age at diagnosis was 40.9 ± 12.2 (range: 12–74) with a male-to-female ratio of 124:87. The average size of CTG repeats was 511.3 (range: 92–1945). Among the DM1 patients with comprehensive clinical data (n = 64, mean age 41.0 ± 12.0), the age at onset was significantly earlier in males than in females (4.8 years earlier, p = 0.026). Muscle weakness (92.2%), myotonia (85.9%), and fatigue (73.4%) were the most prevalent clinical features. The predominant involved muscles at onset are hands (weakness or myotonia) (52.6%) and legs (walking disability) (42.1%). Of them, 70.3% of patients had daytime sleepiness, 14.1% had cataract surgery, 7.8% used wheelchairs, 4.7% required ventilatory support, and 1.6% required gastric tubes. Regarding the comorbidities, 4.7% of patients had tumors, 17.2% had diabetes, 23.4% had dyspnea, 28.1% had intermittent insomnia, 43.8% experienced dysphagia, and 25% exhibited cognitive impairment. Chinese patients exhibited smaller size of CTG repeats (468 ± 139) than those reported in Italy (613 ± 623), the US (629 ± 386), and Japan (625 [302, 1047]), and milder phenotypes with less multisystem involvement. Conclusion The Chinese Han DM1 patients presented milder phenotypes compared to their Caucasian and Japanese counterparts. A male predominance and an early age of onset were identified in male Chinese Han DM1 patients.

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“…Acetylcholine esterase inhibitors are temporarily stopped to reduce bronchial secretions [ 106 ]. IVIG and plasma exchange are regarded as equally effective to treat an MG crisis [ 33 , 107 ]. Up to 80% of the patients respond to both treatments, but there is not a complete overlap between patients responding to the two treatments.…”

Section: Methodsmentioning

confidence: 99%

Myasthenia gravis, respiratory function, and respiratory tract disease

Gilhus

2023

J Neurol

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Myasthenia gravis (MG) is characterized by muscle weakness caused by autoantibodies that bind to the postsynaptic membrane at the neuromuscular junction and impair acetylcholine receptor function. Weakness of respiratory muscles represents the most severe MG manifestation, and 10–15% of all patients experience an MG crisis with the need of mechanical ventilatory support at least once in their life. MG patients with respiratory muscle weakness need active immunosuppressive drug treatment long term, and they need regular specialist follow-up. Comorbidities affecting respiratory function need attention and optimal treatment. Respiratory tract infections can lead to MG exacerbations and precipitate an MG crisis. Intravenous immunoglobulin and plasma exchange are the core treatments for severe MG exacerbations. High-dose corticosteroids, complement inhibitors, and FcRn blockers represent fast-acting treatments that are effective in most MG patients. Neonatal myasthenia is a transient condition with muscle weakness in the newborn caused by mother’s muscle antibodies. In rare cases, treatment of respiratory muscle weakness in the baby is required.

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

Cited by 11 publications

References 35 publications

Pembrolizumab-induced Myopathy with Anti-striated Muscle Antibodies Successfully Treated by Plasma Exchange

Pembrolizumab-induced Myopathy with Anti-striated Muscle Antibodies Successfully Treated by Plasma Exchange

Clinical features and genetic spectrum of a multicenter Chinese cohort with myotonic dystrophy type 1

Myasthenia gravis, respiratory function, and respiratory tract disease

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