AAN Releases Guidelines for IVIG in Some Neuromuscular Diseases

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The American Academy of Neurology (AAN) has released new guidelines on the use of a treatment called intravenous immunoglobulins (IVIG) in various neuromuscular disorders.

The disorders in MDA's program that are included in the guidelines are myasthenia gravis (MG), Lambert-Eaton myasthenic syndrome (LEMS), dermatomyositis (DM), polymyositis (PM) and inclusion-body myositis (IBM).

A patient friendly summary of the guidelines is available on the AAN website.

The AAN guidelines are based on published scientific evidence of the effectiveness of IVIG:

• "strong evidence" means that more than one high-quality scientific study supports IVIG usage;

• "moderate evidence" means at least one high-quality scientific study, or two or more lesser-quality studies, support IVIG;

• "weak evidence" means there are supportive studies, but they are deficient in their design or the strength of their findings; and

• "not enough evidence" means different studies have yielded conflicting results, or there are no studies of reasonable quality.

About IVIG

Immunoglobulins, also known as antibodies, are proteins normally made by the body's immune system to fight infection.

Although it may seem counterintuitive, infusing immunoglobulins taken from pooled blood donations appears to quell an immune response in some circumstances.

In some autoimmune diseases, in which an immune response is misdirected and attacks healthy tissues, intravenous immunoglobulins (IVIG) have apparently helped to "reset" a renegade immune system.

IVIG treatment, however, is not always effective and can be dangerous. For example, it has been associated with swelling around the brain and spinal cord, blood clots, impairment of heart function, kidney failure and hives.

Moderate, weak or insufficient evidence for IVIG in five diseases

In a paper published in Neurology March 27, 2010, a subcommittee of the AAN announced the results of a search of the medical literature published from 1966-2009 on IVIG for neuromuscular disorders.

Based on the evidence from this search, the committee made the following recommendations about five diseases in MDA's program:

• There is moderate evidence showing IVIG can help treat moderate or severe forms of MG. There is not enough evidence to know if IVIG helps in milder forms of MG, and it is important to weigh the benefits and risks of using it in these conditions.

• There is weak evidence showing IVIG may help in the treatment of LEMS.

• There is weak evidence that IVIG may help adults with DM who don’t respond to other therapies.

• There is not enough evidence to support the use of IVIG in PM or IBM.

For more information

• Evidence-Based Guideline: Intravenous Immunoglobulin in the Treatment of Neuromuscular Disorders: Report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology, the scientific paper published in Neurology, March 27, 2012 (read for free);

• IV Immunoglobulin for Treating Neuromuscular Disorders, for a summary of the IVIG guidelines specifically written for patients and their families; and

• Guideline: IVIG Effective for Certain Nerve and Muscle Disorders, for a March 26, 2012, press release on IVIG from the AAN.