Chronic Inflammatory Demyelinating Polyradiculoneuropathy – An Overview of Intravenous Immunoglobulin Therapy

Chronic Inflammatory Demyelinating Polyradiculoneuropathy – An Overview of Intravenous Immunoglobulin Therapy

Brutsaert Dirk L

European Neurological Review, 2009;4(1):72-5

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Abstract
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a significant source of disability, and early diagnosis and immunomodulatory therapy administration are critical to minimise disease progression and axonal degeneration. Intravenous immunoglobulin (IVIg) therapy is considered to be a first-line treatment for CIDP. Comparative short- and long-term data of IVIg versus corticosteroids in CIDP patients are limited. Of the five published placebo-controlled studies in CIDP, four reported only on short-term improvements in disability (≤6 weeks). However, the IGIV CIDP Efficacy (ICE) study, the largest randomised, placebo-controlled CIDP study published to date (n=117), reported significant improvements in disability, functional impairment and quality of life with IVIg (Gamunex®) 1g/kg maintenance therapy every three weeks for up to 48 weeks. Furthermore, long-term IVIg administration was safe and well tolerated, particularly given the short duration of the infusions. Data suggest hat a long-term scheduled maintenance regimen of IVIg in appropriate patients may provide substantial benefit and reduce the risk of CIDP relapse.

Keywords
10% caprylate/chromatography purified, chronic inflammatory demyelinating polyradiculoneuropathy, disability, Gamunex®, efficacy, immune globulin, inflammatory neuropathy cause and treatment, intravenous, safety
Disclosure: Vera Bril has acted as a consultant to Talecris, served on the steering committee of the ICE trial and received unrestricted educational grants for clinical research.
Received: 7 July 2009 Accepted: 1 September 2009
Correspondence: Vera Bril, 13N-1382, Toronto General Hospital, University Health Network, 585 University Avenue, Toronto, Ontario, M5G 2N2, Canada. E: vera.bril@utoronto.ca

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a significant source of disability1 and commonly presents as a progressive, symmetrical weakness in both proximal and distal muscles.2 Underdiagnosis remains a concern, but the prevalence of CIDP worldwide is estimated to be up to eight individuals per 100,000 of the population1,3–8 and it accounts for ~14% of cases of disabling peripheral neuropathies in individuals >65 years of age.9 Long-term prognosis with CIDP is unpredictable in its early stages, and patients may experience a progressive or chronic relapsing course.10 Due to the irreversible damage related to ongoing demyelination and secondary axonal loss in CIDP, early diagnosis and administration of immunomodulatory therapy is critical to minimise further disease progression and axonal degeneration.

Several therapies have been administered in the management of CIDP, with intravenous immunoglobulin (IVIg) therapy and cort costeroids considered first-line treatment options for sensorimotor CIDP.11,12 Furthermore, guidelines recommend IVIg versus corticosteroids as firstline therapy for pure motor CIDP.11 Although the immunomodulatory mechanism of action of IVIg in CIDP has not been fully elucidated, data from other diseases have suggested that IVIg may inhibit autoantibody production, modulate inflammatory mediators and adhesion molecules, induce blockade of Fc receptors (FcRs) on phagocytic cells, alter the activation, differentiation and effector functions of T cells and inhibit complement activation and prevention of membrane attack complex formation.13,14

Dosing and duration of IVIg therapy in clinical practice have previously been based on data from small clinical trials,15,16 with initial treatment of a 2g/kg dose administered over two to five consecutive days.11,12 Maintenance therapy has been recommended for consideration until the maximum benefit has been a chieved and then a dose reduction to find the lowest effective dose.12 However, specific dosing and duration guidelines for maintenance therapy have been lacking, and recommendations have varied from weekly to monthly intervals. Furthermore, long-term published data were limited to non-randomised trials.17–19 In 2008, the IGIV CIDP Efficacy (ICE) study was published, which demonstrated the long-term benefit of IVIg 1g/kg every three weeks as maintenance therapy for CIDP.20 This article will review the efficacy and safety data of IVIg in the treatment of CIDP.

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Keywords:
10% caprylate/chromatography purified, chronic inflammatory demyelinating polyradiculoneuropathy, disability, Gamunex®, efficacy, immune, intravenous immunoglobulin, peripheral nerves, CIDP, inflammatory neuropathy, cidp treatment, intravenous immune globulin, Gamunex immunodeficiency,

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