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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\u2010approved 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\u2010IV. Based on Class I evidence, IVIG is recommended in the treatment of chronic inflammatory demyelinating polyneuropathy, Guillain\u2010Barr\u00e9 Syndrome (GBS) in adults, multifocal motor neuropathy, dermatomyositis, stiff\u2010person syndrome and myasthenia gravis exacerbations but not stable disease. Based on Class II evidence, IVIG is also recommended for Lambert\u2010Eaton myasthenic syndrome and pediatric GBS. In contrast, based on Class I evidence, IVIG is not recommended for inclusion body myositis, post\u2010polio syndrome, IgM paraproteinemic neuropathy and small fiber neuropathy that is idiopathic or associated with tri\u2010sulfated heparin disaccharide or fibroblast growth factor receptor\u20103 autoantibodies. Although only Class IV evidence exists for IVIG use in necrotizing autoimmune myopathy, it should be considered for anti\u2010hydroxy\u20103\u2010methyl\u2010glutaryl\u2010coenzyme A reductase myositis given the risk of long\u2010term disability. Insufficient evidence exists for the use of IVIG in Miller\u2010Fisher syndrome, IgG and IgA paraproteinemic neuropathy, autonomic neuropathy, chronic autoimmune neuropathy, polymyositis, idiopathic brachial plexopathy and diabetic lumbosacral radiculoplexopathy.<\/jats:p>","DOI":"10.1002\/mus.27922","type":"journal-article","created":{"date-parts":[[2023,7,11]],"date-time":"2023-07-11T16:42:44Z","timestamp":1689093764000},"page":"356-374","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":29,"title":["Updated consensus statement: Intravenous immunoglobulin in the treatment of neuromuscular disorders report of the AANEM ad hoc committee"],"prefix":"10.1002","volume":"68","author":[{"given":"Jinny","family":"Tavee","sequence":"first","affiliation":[{"name":"National Jewish Health, Division of Neurology  Denver Colorado USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"suffix":"III","given":"Thomas 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