National Jewish Health, Division of Neurology, Denver, Colorado, USA.
Vagelos College of Physicians and Surgeons, Neurological Institute, Columbia University, New York, New York, USA.
Muscle Nerve. 2023 Oct;68(4):356-374. doi: 10.1002/mus.27922. Epub 2023 Jul 11.
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.
静脉注射免疫球蛋白（IVIG）是一种免疫调节生物疗法，尽管在各种特定疾病中缺乏高质量的证据，但在神经肌肉疾病中的应用越来越多。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。此后，已经有几项关于 IVIG 的随机对照试验，以及新的 FDA 批准的皮肌炎适应症和肌炎修订分类系统，促使 AANEM 召集一个特别小组更新现有的指南。根据对文献的系统回顾更新，新的建议被归类为 I-IV 类。基于 I 类证据，IVIG 推荐用于治疗慢性炎症性脱髓鞘性多发性神经病、成人吉兰-巴雷综合征（GBS）、多灶性运动神经病、皮肌炎、僵人综合征和重症肌无力恶化，但不用于稳定疾病。基于 II 类证据，IVIG 也推荐用于 Lambert-Eaton 肌无力综合征和儿科 GBS。相比之下，基于 I 类证据，IVIG 不推荐用于包涵体肌炎、小儿麻痹后综合征、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。尽管仅存在 IVIG 用于坏死性自身免疫性肌病的 IV 类证据，但鉴于长期残疾的风险，应考虑将其用于抗羟-3-甲基-戊二酰辅酶 A 还原酶肌炎。IVIG 在米勒-费舍尔综合征、IgG 和 IgA 副蛋白血症性神经病、自主神经病、慢性自身免疫性神经病、多发性肌炎、特发性臂丛神经病和糖尿病腰骶神经根神经病中的应用证据不足。
