Intravenous Immune Globulin (IVIg) Therapy

For Neurologic Diseases

  1. Marinos C. Dalakas, MD
+Author Affiliations
  1. From the National Institutes of Health, Bethesda, Maryland. For the current author address, see end
     of text. For definitions of terms used in this article, see glossary at end of text. Acknowledgment: 
    The author thanks B.J. Hessie for skillful editing. Requests for Reprints: Marinos
     C. Dalakas, MD, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Building 10, 
    Room 4N248, 10 Center Drive, MSC 1382, Bethesda, MD 20892-1382.


    High-dose intravenous immune globulin (IVIg) has emerged as an important therapy for various neurologic diseases.
     Different interpretations of clinical trial results; the expected benefit of IVIg compared with that of alternate therapies;
     and issues about IVIg's safety, cost, and mechanisms of action have raised concern and uncertainty among practitioners.
     To clarify these areas, this paper examines the clinical, serologic, and immunologic data on more than 110 patients with various autoimmune neurologic
     diseases who received IVIg during the past 6 years at the National Institute of Neurological Disorders and Stroke. It also reviews work by other investigators 
    on the efficacy, risks, benefits, and mechanisms of the action of IVIg in these diseases.
    In controlled clinical trials, IVIg has been effective in treating the Guillain-Barre syndrome, multifocal motor neuropathy,
     chronic inflammatory demyelinating polyneuropathy, and dermatomyositis. 
    In other controlled or open-label trials and case reports, IVIg produced improvement in several patients with the Lambert-Eaton myasthenic 
    syndrome and myasthenia gravis but had a variable, mild, or unsubstantiated benefit in some patients with inclusion-body myositis, paraproteinemic
     IgM demyelinating polyneuropathy, certain intractable childhood epilepsies, polymyositis, multiple sclerosis, optic neuritis,
     and the stiff-man syndrome. The primary adverse reaction was headache; aseptic meningitis, skin reactions, thromboembolic events, and 
    renal tubular necrosis occurred rarely. The most relevant immunomodulatory actions of IVIg, operating alone or in combination, are inhibition of 
    complement deposition, neutralization of cytokines, modulation of Fc-receptor-mediated phagocytosis, and down-regulation of autoantibody production. 
    Therapy with IVIg is effective for certain autoimmune neurologic diseases, but its spectrum of efficacy has not been fully established. Additional controlled clinical trials are needed.
    By the American College of Physicians

    Intravenous Immunoglobulin in Autoimmune Neuromuscular Diseases

    1. Marinos C. Dalakas, MD
    [+] Author Affiliations
    1. Author Affiliations: Neuromuscular Diseases Section, National Institutes of Health, National Institute of Neurological Disorders and Stroke, Bethesda, Md.


    Context  Intravenous immunoglobulin (IVIG) enhances immune homeostasis by modulating expression and function of Fc 
    receptors, interfering with activation of complement and production of cytokines, providing anti-idiotypic antibodies, and 
    affecting the activation and effector functions of T and B cells. These mechanisms may explain the effectiveness
     of IVIG in autoimmune neuromuscular disorders.
    Objective  To systematically review the current status of the treatment of autoimmune neuromuscular 
    diseases with IVIG, with emphasis on controlled trials.
    Data Sources  Peer-reviewed publications identified through MEDLINE (1966-2003), EMBASE (1974-2003), and
     references from bibliographies of pertinent articles. Each autoimmune neuromuscular disease term was searched 
    in combination with the term intravenous immunoglobulin.
    Study Selection and Data Extraction  Criteria for selection of studies included controlled study design, English
     language, and clinical pertinence. Data quality was based on venue of publication and relevance to clinical care.
    Data Synthesis  Outcomes of controlled trials indicate that IVIG at a total dose of 2 g/kg is effective as first-line
     therapy in Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy, and multifocal motor 
    neuropathy and as second-line therapy in stiff-person syndrome, dermatomyositis, myasthenia gravis, and 
    Lambert-Eaton myasthenic syndrome. In other controlled studies, IVIG produced a modest, variable, and transient
     but not statistically significant benefit in patients with inclusion body myositis and paraproteinemic 
    anti–myelin-associated glycoprotein antibody demyelinating polyneuropathy. Intravenous immunoglobulin is
     not effective in patients with multiple sclerosis who have established weakness or optic neuritis. 
    In myasthenia gravis, it should be reserved for difficult cases or before thymectomy in lieu of plasma exchange.
    Conclusion  Intravenous immunoglobulin is effective in many autoimmune neurologic diseases, but its 
    spectrum of efficacy, especially as first-line therapy, and the appropriate dose for long-term maintenance 
    therapy are not fully established. Further controlled studies of IVIG, combined with a dose-finding 
    effect, pharmacoeconomics, and quality-of-life assessments, are warranted to improve the evidence base

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