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Chronic Inflammatory Demyelinating Polyneuropathy
Chronic Inflammatory Demyelinating Polyradiculoneuropathy –
An Overview of Intravenous Immunoglobulin Therapy
Vera Bril
Division of Neurology, University Health Network, Toronto
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 that 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) Dosing and duration of IVIg therapy in clinical practice have
is a significant source of disability
1
and commonly presents as a previously been based on data from small clinical trials,
15,16
with
progressive, symmetrical weakness in both proximal and distal initial treatment of a 2g/kg dose administered over two to five
muscles.
2
Underdiagnosis remains a concern, but the prevalence of consecutive days.
11,12
Maintenance therapy has been recommended
CIDP worldwide is estimated to be up to eight individuals per 100,000 for consideration until the maximum benefit has been achieved and
of the population
1,3–8
and it accounts for ~14% of cases of disabling then a dose reduction to find the lowest effective dose.
12
However,
peripheral neuropathies in individuals >65 years of age.
9
Long-term specific dosing and duration guidelines for maintenance therapy
prognosis with CIDP is unpredictable in its early stages, and patients have been lacking, and recommendations have varied from weekly
may experience a progressive or chronic relapsing course.
10
Due to to monthly intervals. Furthermore, long-term published data were
the irreversible damage related to ongoing demyelination and limited to non-randomised trials.
17–19
In 2008, the IGIV CIDP Efficacy
secondary axonal loss in CIDP, early diagnosis and administration of (ICE) study was published, which demonstrated the long-term
immunomodulatory therapy is critical to minimise further disease benefit of IVIg 1g/kg every three weeks as maintenance therapy for
progression and axonal degeneration. CIDP.
20
This article will review the efficacy and safety data of IVIg in
the treatment of CIDP.
Several therapies have been administered in the management of CIDP,
with intravenous immunoglobulin (IVIg) therapy and corticosteroids IVIg versus Placebo
considered first-line treatment options for sensorimotor CIDP.
11,12
Five randomised, placebo-controlled trials have been published
Furthermore, guidelines recommend IVIg versus corticosteroids as first- evaluating IVIg versus placebo in the treatment of CIDP,
15,16,20–22
and a
line therapy for pure motor CIDP.
11
Although the immunomodulatory Cochrane systematic review
23
evaluated the pooled data from these
mechanism of action of IVIg in CIDP has not been fully elucidated, data studies (n=235). A significantly higher percentage of patients treated
from other diseases have suggested that IVIg may inhibit autoantibody with IVIg demonstrated an improvement in disability within six weeks
production, modulate inflammatory mediators and adhesion of treatment initiation versus placebo (relative risk [RR] 2.40, 95%
molecules, induce blockade of Fc receptors (FcRs) on phagocytic cells, confidence interval [CI] 1.72–3.36), with a number needed to treat of
alter the activation, differentiation and effector functions of T cells and three to observe an improvement in disability.
23
Four of the five studies
inhibit complement activation and prevention of membrane attack evaluated only short-term improvement in disability (≤6 weeks).
15,16,21,22
complex formation.
13,14
However, the ICE study, the largest randomised, placebo-controlled
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