Consensus statement: The use of intravenous immunoglobulin in the treatment of neuromuscular conditions report of the aanem AD HOC committee

Abstract

Intravenous immunoglobulin (IVIG) is a therapeutic biologic agent that has been prescribed for over two decades to treat various neuromuscular conditions. Most of the treatments are given off-label, as little evidence from large randomized trials exists to support its use. Recently, IGIV-C has received an indication for the treatment of chronic inflammatory demyelinating polyneuropathy (CIDP). Because of the lack of evidence, an ad hoc committee of the AANEM was convened to draft a consensus statement on the rational use of IVIG for neuromuscular disorders. Recommendations were categorized as Class I-IV based on the strength of the medical literature. Class I evidence exists to support the prescription of IVIG to treat patients with Guillain-Barré syndrome (GBS), CIDP, multifocal motor neuropathy, refractory exacerbations of myasthenia gravis, Lambert-Eaton syndrome, dermatomyositis, and stiff person syndrome. Treatment of Fisher syndrome, polymyositis, and certain presumed autoimmune neuromuscular disorders is supported only by Class IV studies, whereas there is no convincing data to substantiate the treatment of inclusion body myopathy (IBM), idiopathic neuropathies, brachial plexopathy, or diabetic amyotrophy using IVIG. Treatment with IVIG must be administered in the context of its known adverse effects. There is little evidence to advise the clinician on the proper dosing of IVIG and duration of therapy.

Descriptive Review and Analysis of Immunoglobulin Utilization Management from 2,548 Prior Authorization Requests

“…[6][7][8][9][10][11][12] These sources are useful in evaluating Ig because the expert opinion and consensus provide balanced evidence assessments. Referencing these publications, we defined the following levels of evidence: "supported," "conditional," "lacking consensus," "currently not supported," and "undetermined."…”

Immunotherapy in Peripheral Neuropathies

“…IVIG remains the treatment of choice for patients with MMN [5,[71][72][73][74][75][76]. IVIG is recommended as a first-line therapy, based on a meta-analysis of 4 randomized, double-blind, placebocontrolled, trials [73][74][75][76], involving a total of 34 patients: 78 % of study participants had a significant short-term improvement in strength, selected as primary outcome measure, following IVIG treatment compared with 4 % following placebo [77].…”

Immune Mediated Diseases and Immune Modulation in the Neurocritical Care Unit

“…SPS is rare, but there has been a single RCT demonstrating a benefit to IVIG therapy; IVIG is thus the therapy with the best available evidence [310,311]. Because SPS is an antibody mediated disorder, it would be expected that plasmapheresis and rituximab would be reasonable treatments, and both have been used with reports of success [309,312].…”

“…In addition, high-dose parenteral corticosteroids are commonly used for treating patients with acute presentations; IVIG and plasmapheresis have also been used with reports of benefit [311,[317][318][319].…”