Intravenous immunoglobulin for chronic inflammatory demyelinating polyradiculoneuropathy: a systematic review

doi:10.1016/S1474-4422(02)00222-3

The Lancet Neurology

Volume 1, Issue 8, December 2002, Pages 491-498

https://doi.org/10.1016/S1474-4422(02)00222-3 Get rights and content

Summary

This review discusses the efficacy and safety in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) of intravenous immunoglobulin and compares this treatment with plasma exchange and prednisolone. We searched publications from 1985 onwards for randomised controlled studies examining the effects of intravenous immunoglobulin in patients with this immune-mediated neuromuscular disorder. Six trials, with 170 patients in total, were judged eligible. A significantly higher proportion of patients improved in disability within a month after the start of treatment with intravenous immunoglobulin than with placebo (relative risk 3·17 [95% CI 1·74 to 5·75]). During this period, intravenous immunoglobulin has similar efficacy to plasma exchange and oral prednisolone; therefore which of these treatments should be the first choice is currently uncertain. An algorithm on treatment approaches for CIDP is proposed.

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 We contacted investigators identified as active on the topic to ask about unpublished or overlooked studies.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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