*仅供医学专业人士阅读参考 用药有一定的应用禁忌及各自特点,要依据患者的个体情况选择 1 2 特别提醒 ▎用法用量: ▎用法用量: 以50mg/d小剂量起始,每隔2~4周增加日剂量50mg,至有效治疗剂量为止(儿童按体重1~2mg/kg·d,成人2~3mg/kg·d,分2~3次口服)。 如无严重和(或)不可耐受的不良反应,可长期服用。主要不良反应包括骨髓抑制(白细胞减少、贫血、血小板减少)、肝功能损害、脱发、流感样症状及消化道症状等,多发生在启动治疗的6周左右。 3 ▎用法用量: 4 按体重400mg/kg·d静脉注射5d。不良反应包括头痛、无菌性脑膜炎、流感样症状和肾功能损害等,故有肾功能损害的患者禁用。 [1]常婷.中国重症肌无力诊断和治疗指南(2020版)[J].中国神经免疫学和神经病学杂志,2021,28(1):1-12. [2]NARAYANASWAMI P,SANDERS D B,WOLFE G,et al.In⁃ternational consensus guidance for management of myasthenia Gravis:2020 update[J].Neurology,2021,96(3):114-122. [3]赵思佳,张慜,任凯夕,等.小剂量利妥昔单抗治疗难治性重症肌无力的长期疗效[J].中国神经免疫学和神经病学杂志,2020,27(5):362-366. [4]HOWARD J F Jr,BAROHN R J,CUTTER G R,et al.A randomized,doubleblind,placebo controlled phase II study of eculizumab in patients with refractory generalized myasthenia gra⁃vis[J].Muscle Nerve,2013,48(1):76-84. [5]HOWARD J F Jr,UTSUGISAWA K,BENATAR M,et al.Safety and efficacy of eculizumab in antiacetylcholine receptor antibodypositive refractory generalised myasthenia gravis(RE⁃GAIN):A phase 3,randomised,double⁃blind,placebo⁃con⁃trolled,multicentre study[J].Lancet Neurol,2017,16(12):976-986. [6]MUPPIDI S,UTSUGISAWA K,BENATAR M,et al.Long⁃term safety and efficacy of eculizumab in generalized myasthenia gravis[J].Muscle Nerve,2019,60(1):14-24. [7]HowardJF,,BrilV,VuT,et al.Safety,efficacy,and tolerability of efgartigimod in patients with generalised myasthenia gravis(ADAPT):a multicentre,randomised,placebo-controlled,phase 3 trial[J].Lancet Neurol,2021,20(7):526-536.DOI:10.1016/s1474-4422(21)00159-9. [8]DÍEZ⁃PORRAS L,HOMEDES C,ALBERTI M A,et al.Intra⁃venous immunoglobulins may prevent prednisone exacerbation in myasthenia gravis[J].Sci Rep,2020,10(1):13497.
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