Purpose To describe the early experience of ravulizumab use in acetylcholine receptor antibody-positive generalized myasthenia gravis (AChR+ve gMG).Methods This multicenter retrospective study included AChR+ve gMG patients who were treated with ravulizumab and had both pre- and post-ravulizumab myasthenia gravis activities of daily living (MG-ADL) scores. Clinical information regarding MG history, concomitant treatment(s), MG-ADL, other MG-specific measures, and adverse events were recorded.Results A total of 18 patients with mean age of 61.83 (+/- 16.08, n = 18) years were included in this cohort. In 10 complement inhibitor naive patients, a clinically meaningful reduction in mean Mg-ADL (baseline: 6.6 (+/- 3.58) vs. 4.4 (+/- 2.28), post ravulizumab) was seen. 6 out of 10 patients (60%) had clinically meaningful reduction post ravulizumab and two achieved minimum symptom expression (MSE). In 8 patients switched from eculizumab to ravulizumab, further reduction was noted in post ravulizumab mean MG-ADL (Baseline: 3.25 (+/- 3.34) vs. 1.5 (+/- 2.34) post ravulizumab). None of the patients who switched from eculizumab to ravulizumab experienced worsening symptoms. Eleven out of 14 (78.5%) patients on prednisone therapy were able to reduce their prednisone dose post-ravulizumab. None of the patients experienced any major side effects.Conclusion In our clinical practice, 60% of AChR+ve gMG complement inhibitor naive patients experienced a clinically meaningful improvement in MG-ADL scores with ravulizumab. Patients were safely switched from eculizumab to ravulizumab and had further improvement in their mean MG-ADL scores. Of those on prednisone therapy, the majority were able to reduce their prednisone dosage.
机构:
Brigham & Womens Hosp, Dept Neurol, Div Neuromuscular Med, Boston, MA 02115 USA
Harvard Med Sch, Boston, MA 02115 USABrigham & Womens Hosp, Dept Neurol, Div Neuromuscular Med, Boston, MA 02115 USA
Suh, Joome
Clarke, Virginia
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Massachusetts Gen Hosp, Dept Neurol, Div Neuromuscular Med, Boston, MA 02114 USABrigham & Womens Hosp, Dept Neurol, Div Neuromuscular Med, Boston, MA 02115 USA
Clarke, Virginia
Amato, Anthony A.
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Brigham & Womens Hosp, Dept Neurol, Div Neuromuscular Med, Boston, MA 02115 USA
Harvard Med Sch, Boston, MA 02115 USABrigham & Womens Hosp, Dept Neurol, Div Neuromuscular Med, Boston, MA 02115 USA
Amato, Anthony A.
Guidon, Amanda C.
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Harvard Med Sch, Boston, MA 02115 USA
Massachusetts Gen Hosp, Dept Neurol, Div Neuromuscular Med, Boston, MA 02114 USABrigham & Womens Hosp, Dept Neurol, Div Neuromuscular Med, Boston, MA 02115 USA
机构:
Tianjin Med Univ, Tianjin Med Univ Gen Hosp, Dept Neurol, Tianjin, Peoples R China
Tianjin Med Univ, Tianjin Med Univ Gen Hosp, Inst Neuroimmunol, Tianjin, Peoples R ChinaTianjin Med Univ, Tianjin Med Univ Gen Hosp, Dept Neurol, Tianjin, Peoples R China
Jia, Dongmei
Zhang, Fenghe
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Tianjin Med Univ, Tianjin Med Univ Gen Hosp, Dept Neurol, Tianjin, Peoples R China
Tianjin Med Univ, Tianjin Med Univ Gen Hosp, Inst Neuroimmunol, Tianjin, Peoples R ChinaTianjin Med Univ, Tianjin Med Univ Gen Hosp, Dept Neurol, Tianjin, Peoples R China
Zhang, Fenghe
Li, Huining
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Tianjin Med Univ, Tianjin Med Univ Gen Hosp, Dept Neurol, Tianjin, Peoples R China
Tianjin Med Univ, Tianjin Med Univ Gen Hosp, Inst Neuroimmunol, Tianjin, Peoples R ChinaTianjin Med Univ, Tianjin Med Univ Gen Hosp, Dept Neurol, Tianjin, Peoples R China
Li, Huining
Shen, Yi
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Tianjin Med Univ, Tianjin Med Univ Gen Hosp, Dept Neurol, Tianjin, Peoples R China
Tianjin Med Univ, Tianjin Med Univ Gen Hosp, Inst Neuroimmunol, Tianjin, Peoples R ChinaTianjin Med Univ, Tianjin Med Univ Gen Hosp, Dept Neurol, Tianjin, Peoples R China
Shen, Yi
Jin, Zhao
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Third Cent Hosp Tianjin, Dept Neurol, Tianjin, Peoples R ChinaTianjin Med Univ, Tianjin Med Univ Gen Hosp, Dept Neurol, Tianjin, Peoples R China
Jin, Zhao
Shi, Fu-Dong
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Tianjin Med Univ, Tianjin Med Univ Gen Hosp, Dept Neurol, Tianjin, Peoples R China
Tianjin Med Univ, Tianjin Med Univ Gen Hosp, Inst Neuroimmunol, Tianjin, Peoples R ChinaTianjin Med Univ, Tianjin Med Univ Gen Hosp, Dept Neurol, Tianjin, Peoples R China
Shi, Fu-Dong
Zhang, Chao
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Tianjin Med Univ, Tianjin Med Univ Gen Hosp, Dept Neurol, Tianjin, Peoples R China
Tianjin Med Univ, Tianjin Med Univ Gen Hosp, Inst Neuroimmunol, Tianjin, Peoples R ChinaTianjin Med Univ, Tianjin Med Univ Gen Hosp, Dept Neurol, Tianjin, Peoples R China
机构:
Sun Yat Sen Univ, Affiliated Hosp 1, Dept Neurol, Guangzhou, Peoples R China
Natl Key Clin Dept, Guangdong Prov Key Lab Diag & Treatment Major Neu, Guangzhou, Peoples R China
Key Discipline Neurol, Guangzhou, Peoples R ChinaSun Yat Sen Univ, Affiliated Hosp 1, Dept Neurol, Guangzhou, Peoples R China
Chen, Jiaxin
Li, Shiyin
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Sun Yat Sen Univ, Affiliated Hosp 1, Dept Neurol, Guangzhou, Peoples R China
Natl Key Clin Dept, Guangdong Prov Key Lab Diag & Treatment Major Neu, Guangzhou, Peoples R China
Key Discipline Neurol, Guangzhou, Peoples R ChinaSun Yat Sen Univ, Affiliated Hosp 1, Dept Neurol, Guangzhou, Peoples R China
Li, Shiyin
Feng, Li
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Sun Yat Sen Univ, Affiliated Hosp 1, Dept Neurol, Guangzhou, Peoples R China
Natl Key Clin Dept, Guangdong Prov Key Lab Diag & Treatment Major Neu, Guangzhou, Peoples R China
Key Discipline Neurol, Guangzhou, Peoples R ChinaSun Yat Sen Univ, Affiliated Hosp 1, Dept Neurol, Guangzhou, Peoples R China
Feng, Li
Wang, Haiyan
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Sun Yat Sen Univ, Affiliated Hosp 1, Dept Neurol, Guangzhou, Peoples R China
Natl Key Clin Dept, Guangdong Prov Key Lab Diag & Treatment Major Neu, Guangzhou, Peoples R China
Key Discipline Neurol, Guangzhou, Peoples R ChinaSun Yat Sen Univ, Affiliated Hosp 1, Dept Neurol, Guangzhou, Peoples R China
Wang, Haiyan
Huang, Xin
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Sun Yat Sen Univ, Affiliated Hosp 1, Dept Neurol, Guangzhou, Peoples R China
Natl Key Clin Dept, Guangdong Prov Key Lab Diag & Treatment Major Neu, Guangzhou, Peoples R China
Key Discipline Neurol, Guangzhou, Peoples R ChinaSun Yat Sen Univ, Affiliated Hosp 1, Dept Neurol, Guangzhou, Peoples R China
Huang, Xin
Feng, Huiyu
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Sun Yat Sen Univ, Affiliated Hosp 1, Dept Neurol, Guangzhou, Peoples R China
Natl Key Clin Dept, Guangdong Prov Key Lab Diag & Treatment Major Neu, Guangzhou, Peoples R China
Key Discipline Neurol, Guangzhou, Peoples R ChinaSun Yat Sen Univ, Affiliated Hosp 1, Dept Neurol, Guangzhou, Peoples R China