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Meta-Analysis
. 2024 Feb 14;2(2):CD001797.
doi: 10.1002/14651858.CD001797.pub4.

Intravenous immunoglobulin for chronic inflammatory demyelinating polyradiculoneuropathy

Affiliations
Meta-Analysis

Intravenous immunoglobulin for chronic inflammatory demyelinating polyradiculoneuropathy

Sander Rm Bus et al. Cochrane Database Syst Rev. .

Abstract

Background: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) causes progressive or relapsing weakness and numbness of the limbs, which lasts for at least two months. Uncontrolled studies have suggested that intravenous immunoglobulin (IVIg) could help to reduce symptoms. This is an update of a review first published in 2002 and last updated in 2013.

Objectives: To assess the efficacy and safety of intravenous immunoglobulin in people with chronic inflammatory demyelinating polyradiculoneuropathy.

Search methods: We searched the Cochrane Neuromuscular Specialised Register, CENTRAL, MEDLINE, Embase, and two trials registers on 8 March 2023.

Selection criteria: We selected randomised controlled trials (RCTs) and quasi-RCTs that tested any dose of IVIg versus placebo, plasma exchange, or corticosteroids in people with definite or probable CIDP.

Data collection and analysis: We used standard Cochrane methods. Our primary outcome was significant improvement in disability within six weeks after the start of treatment, as determined and defined by the study authors. Our secondary outcomes were change in mean disability score within six weeks, change in muscle strength (Medical Research Council (MRC) sum score) within six weeks, change in mean disability score at 24 weeks or later, frequency of serious adverse events, and frequency of any adverse events. We used GRADE to assess the certainty of evidence for our main outcomes.

Main results: We included nine RCTs with 372 participants (235 male) from Europe, North America, South America, and Israel. There was low statistical heterogeneity between the trial results, and the overall risk of bias was low for all trials that contributed data to the analysis. Five trials (235 participants) compared IVIg with placebo, one trial (20 participants) compared IVIg with plasma exchange, two trials (72 participants) compared IVIg with prednisolone, and one trial (45 participants) compared IVIg with intravenous methylprednisolone (IVMP). We included one new trial in this update, though it contributed no data to any meta-analyses. IVIg compared with placebo increases the probability of significant improvement in disability within six weeks of the start of treatment (risk ratio (RR) 2.40, 95% confidence interval (CI) 1.72 to 3.36; number needed to treat for an additional beneficial outcome (NNTB) 4, 95% CI 3 to 5; 5 trials, 269 participants; high-certainty evidence). Since each trial used a different disability scale and definition of significant improvement, we were unable to evaluate the clinical relevance of the pooled effect. IVIg compared with placebo improves disability measured on the Rankin scale (0 to 6, lower is better) two to six weeks after the start of treatment (mean difference (MD) -0.26 points, 95% CI -0.48 to -0.05; 3 trials, 90 participants; high-certainty evidence). IVIg compared with placebo probably improves disability measured on the Inflammatory Neuropathy Cause and Treatment (INCAT) scale (1 to 10, lower is better) after 24 weeks (MD 0.80 points, 95% CI 0.23 to 1.37; 1 trial, 117 participants; moderate-certainty evidence). There is probably little or no difference between IVIg and placebo in the frequency of serious adverse events (RR 0.82, 95% CI 0.36 to 1.87; 3 trials, 315 participants; moderate-certainty evidence). The trial comparing IVIg with plasma exchange reported none of our main outcomes. IVIg compared with prednisolone probably has little or no effect on the probability of significant improvement in disability four weeks after the start of treatment (RR 0.91, 95% CI 0.50 to 1.68; 1 trial, 29 participants; moderate-certainty evidence), and little or no effect on change in mean disability measured on the Rankin scale (MD 0.21 points, 95% CI -0.19 to 0.61; 1 trial, 24 participants; moderate-certainty evidence). There is probably little or no difference between IVIg and prednisolone in the frequency of serious adverse events (RR 0.45, 95% CI 0.04 to 4.69; 1 cross-over trial, 32 participants; moderate-certainty evidence). IVIg compared with IVMP probably increases the likelihood of significant improvement in disability two weeks after starting treatment (RR 1.46, 95% CI 0.40 to 5.38; 1 trial, 45 participants; moderate-certainty evidence). IVIg compared with IVMP probably has little or no effect on change in disability measured on the Rankin scale two weeks after the start of treatment (MD 0.24 points, 95% CI -0.15 to 0.63; 1 trial, 45 participants; moderate-certainty evidence) or on change in mean disability measured with the Overall Neuropathy Limitation Scale (ONLS, 1 to 12, lower is better) 24 weeks after the start of treatment (MD 0.03 points, 95% CI -0.91 to 0.97; 1 trial, 45 participants; moderate-certainty evidence). The frequency of serious adverse events may be higher with IVIg compared with IVMP (RR 4.40, 95% CI 0.22 to 86.78; 1 trial, 45 participants, moderate-certainty evidence).

Authors' conclusions: Evidence from RCTs shows that IVIg improves disability for at least two to six weeks compared with placebo, with an NNTB of 4. During this period, IVIg probably has similar efficacy to oral prednisolone and IVMP. Further placebo-controlled trials are unlikely to change these conclusions. In one large trial, the benefit of IVIg compared with placebo in terms of improved disability score persisted for 24 weeks. Further research is needed to assess the long-term benefits and harms of IVIg relative to other treatments.

Trial registration: ClinicalTrials.gov NCT01349270 NCT02638207 NCT01225276 NCT03684018.

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Conflict of interest statement

SB: reports no competing interests. RdH: reports no competing interests. MV: reports no competing interests. INvS: chairs a steering committee for CSL‐Behring and received departmental honoraria for serving on scientific advisory boards for CSL‐Behring and UCB. He received speakers' fees from CSL‐Behring. Departmental research support has been granted by The Netherlands Organisation for Scientific Research, and the Dutch Prinses Beatrix Spierfonds. All lecturing and consulting fees for INvS were donated to the Stichting Klinische Neurologie, a local foundation that supports research in the field of neurological disorders. INvS is a member of the Scientific Board of the Kreuth III meeting on the optimal use of plasma‐derived medicinal products, especially coagulation factors and normal immunoglobulins organised under the auspices of the European Directorate for the Quality of Medicines & HealthCare (EDQM). FE: received support for printing of his thesis from Sanquin Bloedvoorziening (Dutch blood bank and IVIg manufacturer) and CSL‐Behring in 2014. He reports lecture fees from CSL‐Behring, Kedrion, and Grifols. Outside the submitted work, as principal investigator of INCbase, FE also reports investigator‐initiated grants from Kedrion, Terumo BCT, CSL‐Behring, and Takeda Pharmaceutical Company, and grants from ZonMw (Dutch governmental agency) and Prinses Beatrix Spierfonds (a Dutch charity). In addition, his institution has received fees from UCB Pharma, CSL‐Behring, and Takeda for advisory board membership. All grants and fees were paid to his institution. He is a member of the Cochrane Neuromuscular Editorial Board.

Figures

1
1
Study flow diagram for the current update.
2
2
Review authors' judgements about each risk of bias item for each included study. Green: low risk of bias; yellow: unclear risk of bias; red: high risk of bias.
1.1
1.1. Analysis
Comparison 1: Intravenous immunoglobulin (IVIg) versus placebo, Outcome 1: Significant improvement in disability within 6 weeks
1.2
1.2. Analysis
Comparison 1: Intravenous immunoglobulin (IVIg) versus placebo, Outcome 2: Significant improvement in disability within 6 weeks; generic inverse variance approach
1.3
1.3. Analysis
Comparison 1: Intravenous immunoglobulin (IVIg) versus placebo, Outcome 3: Improvement ≥ 1 point on Rankin scale
1.4
1.4. Analysis
Comparison 1: Intravenous immunoglobulin (IVIg) versus placebo, Outcome 4: Change in mean disability score within 6 weeks
1.5
1.5. Analysis
Comparison 1: Intravenous immunoglobulin (IVIg) versus placebo, Outcome 5: Change in mean disability score within 6 weeks; generic inverse variance approach
1.6
1.6. Analysis
Comparison 1: Intravenous immunoglobulin (IVIg) versus placebo, Outcome 6: Change in mean disability score on Rankin scale within 6 weeks
1.7
1.7. Analysis
Comparison 1: Intravenous immunoglobulin (IVIg) versus placebo, Outcome 7: Change in muscle strength (mean Medical Research Council sum score) within 6 weeks
1.8
1.8. Analysis
Comparison 1: Intravenous immunoglobulin (IVIg) versus placebo, Outcome 8: Change in mean disability score at 24 weeks or later
1.9
1.9. Analysis
Comparison 1: Intravenous immunoglobulin (IVIg) versus placebo, Outcome 9: Frequency of serious side effects
1.10
1.10. Analysis
Comparison 1: Intravenous immunoglobulin (IVIg) versus placebo, Outcome 10: Frequency of any side effects
2.1
2.1. Analysis
Comparison 2: Intravenous immunoglobulin (IVIg) versus plasma exchange (PE), Outcome 1: Change in mean disability score within 6 weeks
3.1
3.1. Analysis
Comparison 3: Intravenous immunoglobulin (IVIg) versus prednisolone, Outcome 1: Significant improvement in disability within 6 weeks
3.2
3.2. Analysis
Comparison 3: Intravenous immunoglobulin (IVIg) versus prednisolone, Outcome 2: Improvement of ≥ 1 point on Rankin scale
3.3
3.3. Analysis
Comparison 3: Intravenous immunoglobulin (IVIg) versus prednisolone, Outcome 3: Change in mean disability score within 6 weeks
3.4
3.4. Analysis
Comparison 3: Intravenous immunoglobulin (IVIg) versus prednisolone, Outcome 4: Change in mean disability score on Rankin scale within 6 weeks
3.5
3.5. Analysis
Comparison 3: Intravenous immunoglobulin (IVIg) versus prednisolone, Outcome 5: Change in muscle strength (mean Medical Research Council sum score) within 6 weeks
3.6
3.6. Analysis
Comparison 3: Intravenous immunoglobulin (IVIg) versus prednisolone, Outcome 6: Frequency of serious side effects
3.7
3.7. Analysis
Comparison 3: Intravenous immunoglobulin (IVIg) versus prednisolone, Outcome 7: Frequency of any side effects
4.1
4.1. Analysis
Comparison 4: Intravenous immunoglobulin (IVIg) versus intravenous methylprednisolone (IVMP), Outcome 1: Significant improvement in disability within 6 weeks
4.2
4.2. Analysis
Comparison 4: Intravenous immunoglobulin (IVIg) versus intravenous methylprednisolone (IVMP), Outcome 2: Improvement of ≥ 1 point on Rankin scale
4.3
4.3. Analysis
Comparison 4: Intravenous immunoglobulin (IVIg) versus intravenous methylprednisolone (IVMP), Outcome 3: Change in mean disability score within 6 weeks
4.4
4.4. Analysis
Comparison 4: Intravenous immunoglobulin (IVIg) versus intravenous methylprednisolone (IVMP), Outcome 4: Change in mean disability score on Rankin scale within 6 weeks
4.5
4.5. Analysis
Comparison 4: Intravenous immunoglobulin (IVIg) versus intravenous methylprednisolone (IVMP), Outcome 5: Change in muscle strength (Medical Research Council sum score) within 6 weeks
4.6
4.6. Analysis
Comparison 4: Intravenous immunoglobulin (IVIg) versus intravenous methylprednisolone (IVMP), Outcome 6: Change in mean disability score at 24 weeks or later
4.7
4.7. Analysis
Comparison 4: Intravenous immunoglobulin (IVIg) versus intravenous methylprednisolone (IVMP), Outcome 7: Frequency of serious side effects
4.8
4.8. Analysis
Comparison 4: Intravenous immunoglobulin (IVIg) versus intravenous methylprednisolone (IVMP), Outcome 8: Frequency of any side effects

Update of

References

References to studies included in this review

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References to other published versions of this review

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