New AAN Guideline on Plasmapheresis in Neurologic Disorders

Susan Jeffrey

January 21, 2011

January 21, 2011 — The American Academy of Neurology (AAN) has issued new guidelines on the use of plasmapheresis in the treatment of neurologic disorders.

The guidelines provide evidence-based assessment of the use of this approach, also called therapeutic plasma exchange, and now endorses it for treatment of severe relapses in multiple sclerosis (MS), treatment of severe acute inflammatory demyelinating polyneuropathy (AIDP)/Guillain-Barré syndrome (GBS), and short-term management of chronic inflammatory demyelinating polyneuropathy (CIDP).

They recommend against its use in chronic or secondary progressive MS and conclude that, despite its frequent use to manage myasthenic crises associated with myasthenia gravis, there is still insufficient evidence to establish its efficacy.

Dr. Vinay Chaudhry

Vinay Chaudhry, MD, from Johns Hopkins University School of Medicine, Baltimore, Maryland, and a member of the writing group, said the new guidelines focus not on the technique of plasmapheresis per se but rather in which clinical settings it should be considered.

Many neurological disorders have few effective treatments and management tends to be symptomatic, Dr. Chaudhry told Medscape Medical News. "But the ones that are more treatable are the so-called immune-mediated disorders, and there are several of them that we've looked at in this update."

The document, prepared for the AAN Therapeutics and Technology Assessment subcommittee, was published in the January 18 issue of Neurology. It has been endorsed by the American Association of Neuromuscular and Electrodiagnostic Medicine, and the National Multiple Sclerosis Society endorsed the section of the guideline relating to the use of plasmapheresis in MS.

Recommendations

The mechanics of plasma exchange has not changed since the introduction of continuous flow machines, the study authors write, but the guidelines have not been updated since 1996. The previous guideline also did not use the academy's current "stringent" evidence-based approach, said Dr. Chaudhry.

They divided their deliberations into conditions of the central and peripheral nervous system.

Among their conclusions:

  • Plasmapheresis is established as effective and should be offered in severe AIDP/GBS (Class I studies, Level A). "There were no new studies, but the previous studies were relooked at with this class evidence, and it was clear that if you have a severe Guillain-Barre — meaning that the person comes in with rapidly progressive weakness to the point that they're not able to walk, and you use plasma exchange, they improve more quickly with the plasma exchange than without," Dr. Chaudhry said.

  • Plasmapheresis is probably effective and should be considered for mild AIDP/GBS (Level B). "The level of evidence was not as strong, but it's probably effective in that group," he added. "That's suggestive that perhaps one should look into more research."

  • Plasmapheresis is established as effective and should be offered in the short-term management of CIDP (Class I studies, Level A). CIDP can be a long-standing disease, but in that group it's an established and effective treatment, he noted. "The question is how long would the treatment last and because that's a chronic immune disease, this group did not address that, so that's another research topic."

  • It is probably effective and should be considered as second-line treatment of steroid-resistant exacerbations in relapsing forms of MS, based on at least 1 Class I or 2 Class II studies (Level B).

  • Plasmapheresis is established as ineffective and should not be offered for chronic or secondary progressive MS (Class I studies, Level A).

  • Plasmapheresis is probably effective and should be considered for neuropathy associated with IgA or IgG gammopathy, based on at least 1 Class I or 2 Class II studies (Level B).

  • This approach is probably not effective and should not be considered for neuropathy associated with IgM gammopathy, based on 1 Class I study (Level B).

  • Plasmapheresis is possibly effective and may be considered for acute fulminant demyelinating CNS disease (Level C).

  • There is insufficient evidence to support or refute the use of plasmapheresis for myasthenia gravis (Class III evidence, Level U). This finding, "came as a surprise, because most neurologists use plasma exchange for at least the myasthenic crisis, but in this study the evidence is not conclusive," Dr. Chaudhry said. "The evidence we had was Class III, so it's labeled insufficient evidence, but neurologists are using this treatment either in preparation for a thymectomy for patients who have myasthenia gravis or in the crisis situation, so maybe another study to prove that benefit is in order."

  • Finally, they conclude that there is insufficient evidence to support or refute the use of plasmapheresis pediatric autoimmune neuropsychiatric disorders associated with streptococcus infection (PANDAS) and Sydenham chorea (Class III evidence, Level U).

Dr. Chaudhry serves on the editorial board of Neurologist; is an inventor on patent(s) re: Total Neuropathy Score (TNS)—a score for evaluating peripheral neuropathies, for which he receives technology royalties from Abbott, Johnson & Johnson, and sanofi-aventis; receives publishing royalties for Harrison's Principles of Internal Medicine, 17th ed (McGraw Hill Companies Inc, 2008); estimates that 40% of his clinical effort is spent on nerve conduction studies; has given expert testimony for the Department of Health and Human Services Vaccine Injury Compensation program; and receives research support from the Neuropathy Association, Nutricia, and Insmed Inc. Disclosures for other members of the writing group appear in the article.

Neurology. 2011;76:294-300.

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