Abstract：
Intravenous immune globulin (IVIG) is an immune-modulating biologic therapy that is increasingly being used in neuromuscular disorders despite the paucity of high-quality evidence for various specific diseases. To address this, the AANEM created the 2009 consensus statement to provide guidance on the use of IVIG in neuromuscular disorders. Since then, there have been several randomized controlled trials for IVIG, a new FDA-approved indication for dermatomyositis and a revised classification system for myositis, prompting the AANEM to convene an ad hoc panel to update the existing guidelines.New recommendations based on an updated systemic review of the literature were categorized as Class I-IV. Based on Class I evidence, IVIG is recommended in the treatment of chronic inflammatory demyelinating polyneuropathy, Guillain-Barré Syndrome (GBS) in adults, multifocal motor neuropathy, dermatomyositis, stiff-person syndrome and myasthenia gravis exacerbations but not stable disease. Based on Class II evidence, IVIG is also recommended for Lambert-Eaton myasthenic syndrome and pediatric GBS. In contrast, based on Class I evidence, IVIG is not recommended for inclusion body myositis, post-polio syndrome, IgM paraproteinemic neuropathy and small fiber neuropathy that is idiopathic or associated with tri-sulfated heparin disaccharide or fibroblast growth factor receptor-3 autoantibodies. Although only Class IV evidence exists for IVIG use in necrotizing autoimmune myopathy, it should be considered for anti-hydroxy-3-methyl-glutaryl-coenzyme A reductase myositis given the risk of long-term disability. Insufficient evidence exists for the use of IVIG in Miller-Fisher syndrome, IgG and IgA paraproteinemic neuropathy, autonomic neuropathy, chronic autoimmune neuropathy, polymyositis, idiopathic brachial plexopathy and diabetic lumbosacral radiculoplexopathy.
摘要：
静脉内免疫球蛋白（IVIG）是一种免疫调节生物疗法，尽管缺乏各种特定疾病的高质量证据，但越来越多地用于神经肌肉疾病。为了解决这个问题，AANEM创建了2009年共识声明,为IVIG在神经肌肉疾病中的应用提供指导.从那以后,已经有几个针对IVIG的随机对照试验，FDA批准的皮肌炎新适应症和修订的肌炎分类系统，提示AANEM召集一个特设小组来更新现有指南。基于文献更新的系统综述的新建议被归类为I-IV类。根据第一类证据,IVIG被推荐用于治疗慢性炎症性脱髓鞘性多发性神经病，格林-巴利综合征（GBS）在成人，多灶性运动神经病,皮肌炎,僵硬人综合征和重症肌无力恶化，但不是稳定的疾病。根据二类证据,IVIG也推荐用于Lambert-Eaton肌无力综合征和小儿GBS。相比之下,根据第一类证据,IVIG不推荐用于包涵体肌炎，脊髓灰质炎后综合征,IgM副蛋白血症性神经病和特发性或与三硫酸肝素二糖或成纤维细胞生长因子受体3自身抗体相关的小纤维神经病。虽然只有IV类证据表明IVIG用于坏死性自身免疫性肌病，考虑到长期残疾的风险,应考虑抗羟基-3-甲基-戊二酰辅酶A还原酶肌炎.在Miller-Fisher综合征中使用IVIG的证据不足，IgG和IgA副蛋白血症神经病,自主神经病变,慢性自身免疫性神经病,多发性肌炎,特发性臂丛神经病变和糖尿病腰骶部神经丛病变。
