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.