ReviewIntravenous immunoglobulin for chronic inflammatory demyelinating polyradiculoneuropathy: a systematic review
Section snippets
Literature search
We searched without language restriction the Cochrane Neuromuscular Disease Review Group trial register, Medline (from 1985), EMBASE (from 1985), and ISI (from 1985) for clinical trials on the effect of intravenous immunoglobulin in CIDP. We also searched the references listed in the published studies, reviews, textbooks, and relevant conference proceedings. We contacted investigators identified as active on the topic to ask about unpublished or overlooked studies.
Study selection and assessment of methodological quality
We selected for inclusion
Diagnosis
CIDP is characterised by motor, sensory, or both symptoms and signs in more than one limb, developing over at least 2 months.18, 19, 20, 21 The disease runs a progressive, relapsing—remitting, or monophasic course. Sensory disturbances are generally slight but are found in about 80% of patients. Predominantly motor involvement occurs in 10–20%; predominantly sensory involvement has been reported but is rare.22, 23 Proximal and distal parts of the limbs are affected symmetrically in most cases.
Description of studies
The search strategy identified six randomised controlled trials that were judged eligible; they included a total of 170 patients with CIDP.36, 37, 38, 39, 40, 41 In four trials intravenous immunoglobulin treatment was compared with placebo,36, 37, 38, 39 in one it was compared with plasma exchange,40 and in one it was compared with prednisolone.41 Two studies had a parallel-group design,37, 39 and the others had a cross-over design. A total of 2·0 g/kg bodyweight of intravenous immunoglobulin
A treatment algorithm for CIDP
Intravenous immunoglobulin, plasma exchange, and prednisolone treatment have been sufficiently investigated to be incorporated into a guideline. However, information on exact treatment protocols (dose, taper regimen, frequency of administration) is incomplete. The information here is largely based on our interpretation of published studies and our personal experience. A treatment algorithm, which we use for patients with CIDP, is shown in figure 3.
The future
A recent pilot study in patients with CIDP of six cycles of pulsed high-dose dexamethasone administered orally for 4 days every 4 weeks showed that the regimen was effective and well tolerated and induced remissions in six of ten patients during a mean of 20 months (SD 3); seven patients improved in functional status.60 Of the four newly diagnosed, previously untreated patients, three reached remission. Five patients experienced side-effects during dexamethasone treatment, which were all
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Cited by (50)
Update on the use of immunoglobulin in human disease: A review of evidence
2017, Journal of Allergy and Clinical ImmunologyA retrospective study on the efficacy and safety of intraveinous immunoglobulin (Tegeline<sup>®</sup>) in patients with chronic inflammatory demyelinating polyneuropathy
2015, Presse MedicaleCitation Excerpt :Efficacy at 12 months could not be analyzed because of missing data (modified Rankin score data for 5 patients and investigators’ overall assessment for 8 patients). The sample size was calculated on the basis of the expected responder rate with IV Ig (47%) and with placebo (15%), estimated based on a meta-analysis by van Schaik et al. [28]. The ICE study [19], which was published after the meta-analysis, reported two placebo responder rates of 21% and 22% and a responder rate with IVIg of 56%.
Chronic inflammatory demyelinating polyneuropathy
2013, Handbook of Clinical NeurologyChronic inflammatory demyelinating polyradiculoneuropathy
2010, Revue de Medecine InternePulsed high-dose dexamethasone versus standard prednisolone treatment for chronic inflammatory demyelinating polyradiculoneuropathy (PREDICT study): a double-blind, randomised, controlled trial
2010, The Lancet NeurologyCitation Excerpt :By use of results from pilot studies, we estimated that at least 67% (95% CI 39–88%) of newly diagnosed patients with CIDP who were treatment naive would reach remission if treated with dexamethasone. Previous studies summarised in a Cochrane review have shown that spontaneous remissions occur in 15% (95% CI 7–25%) of patients with CIDP.29 Thus, we assumed that the proportion of patients with spontaneous remission was unlikely to be higher than 25%.
This paper is based partly on a more detailed Cochrane review published in the Cochrane Library, Issue 2, 2002. Cochrane reviews are regularly updated as new evidence emerges and in response to comments and criticisms, and the Cochrane Library should be consulted for the most recent version of the review.