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. 2017 Jan 13;1(1):CD010369.
doi: 10.1002/14651858.CD010369.pub2.

Treatments for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): an overview of systematic reviews

Anne Louise Oaklander  1 , Michael Pt Lunn  2 , Richard Ac Hughes  3 , Ivo N van Schaik  4 , Chris Frost  5 , Colin H Chalk  6
Affiliations

Treatments for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): an overview of systematic reviews

Anne Louise Oaklander et al. Cochrane Database Syst Rev. .

Abstract

Background: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic progressive or relapsing and remitting disease that usually causes weakness and sensory loss. The symptoms are due to autoimmune inflammation of peripheral nerves. CIPD affects about 2 to 3 per 100,000 of the population. More than half of affected people cannot walk unaided when symptoms are at their worst. CIDP usually responds to treatments that reduce inflammation, but there is disagreement about which treatment is most effective.

Objectives: To summarise the evidence from Cochrane systematic reviews (CSRs) and non-Cochrane systematic reviews of any treatment for CIDP and to compare the effects of treatments.

Methods: We considered all systematic reviews of randomised controlled trials (RCTs) of any treatment for any form of CIDP. We reported their primary outcomes, giving priority to change in disability after 12 months.Two overview authors independently identified published systematic reviews for inclusion and collected data. We reported the quality of evidence using GRADE criteria. Two other review authors independently checked review selection, data extraction and quality assessments.On 31 October 2016, we searched the Cochrane Database of Systematic Reviews, the Database of Abstracts of Reviews of Effects (in theCochrane Library), MEDLINE, Embase, and CINAHL Plus for systematic reviews of CIDP. We supplemented the RCTs in the existing CSRs by searching on the same date for RCTs of any treatment of CIDP (including treatment of fatigue or pain in CIDP), in the Cochrane Neuromuscular Specialised Register, CENTRAL, MEDLINE, Embase, and CINAHL Plus.

Main results: Five CSRs met our inclusion criteria. We identified 23 randomised trials, of which 15 had been included in these CSRs. We were unable to compare treatments as originally planned, because outcomes and outcome intervals differed. CorticosteroidsIt is uncertain whether daily oral prednisone improved impairment compared to no treatment because the quality of the evidence was very low (1 trial, 28 participants). According to moderate-quality evidence (1 trial, 41 participants), six months' treatment with high-dose monthly oral dexamethasone did not improve disability more than daily oral prednisolone. Observational studies tell us that prolonged use of corticosteroids sometimes causes serious side-effects. Plasma exchangeAccording to moderate-quality evidence (2 trials, 59 participants), twice-weekly plasma exchange produced more short-term improvement in disability than sham exchange. In the largest observational study, 3.9% of plasma exchange procedures had complications. Intravenous immunoglobulinAccording to high-quality evidence (5 trials, 269 participants), intravenous immunoglobulin (IVIg) produced more short-term improvement than placebo. Adverse events were more common with IVIg than placebo (high-quality evidence), but serious adverse events were not (moderate-quality evidence, 3 trials, 315 participants). One trial with 19 participants provided moderate-quality evidence of little or no difference in short-term improvement of impairment with plasma exchange in comparison to IVIg. There was little or no difference in short-term improvement of disability with IVIg in comparison to oral prednisolone (moderate-quality evidence; 1 trial, 29 participants) or intravenous methylprednisolone (high-quality evidence; 1 trial, 45 participants). One unpublished randomised open trial with 35 participants found little or no difference in disability after three months of IVIg compared to oral prednisone; this trial has not yet been included in a CSR. We know from observational studies that serious adverse events related to IVIg do occur. Other immunomodulatory treatmentsIt is uncertain whether the addition of azathioprine (2 mg/kg) to prednisone improved impairment in comparison to prednisone alone, as the quality of the evidence is very low (1 trial, 27 participants). Observational studies show that adverse effects truncate treatment in 10% of people.According to low-quality evidence (1 trial, 60 participants), compared to placebo, methotrexate 15 mg/kg did not allow more participants to reduce corticosteroid or IVIg doses by 20%. Serious adverse events were no more common with methotrexate than with placebo, but observational studies show that methotrexate can cause teratogenicity, abnormal liver function, and pulmonary fibrosis.According to moderate-quality evidence (2 trials, 77 participants), interferon beta-1a (IFN beta-1a) in comparison to placebo, did not allow more people to withdraw from IVIg. According to moderate-quality evidence, serious adverse events were no more common with IFN beta-1a than with placebo.We know of no other completed trials of immunosuppressant or immunomodulatory agents for CIDP. Other treatmentsWe identified no trials of treatments for fatigue or pain in CIDP. Adverse effectsNot all trials routinely collected adverse event data; when they did, the quality of evidence was variable. Adverse effects in the short, medium, and long term occur with all interventions. We are not able to make reliable comparisons of adverse events between the interventions included in CSRs.

Authors' conclusions: We cannot be certain based on available evidence whether daily oral prednisone improves impairment compared to no treatment. However, corticosteroids are commonly used, based on widespread availability, low cost, very low-quality evidence from observational studies, and clinical experience. The weakness of the evidence does not necessarily mean that corticosteroids are ineffective. High-dose monthly oral dexamethasone for six months is probably no more or less effective than daily oral prednisolone. Plasma exchange produces short-term improvement in impairment as determined by neurological examination, and probably produces short-term improvement in disability. IVIg produces more short-term improvement in disability than placebo and more adverse events, although serious side effects are probably no more common than with placebo. There is no clear difference in short-term improvement in impairment with IVIg when compared with intravenous methylprednisolone and probably no improvement when compared with either oral prednisolone or plasma exchange. According to observational studies, adverse events related to difficult venous access, use of citrate, and haemodynamic changes occur in 3% to17% of plasma exchange procedures.It is uncertain whether azathioprine is of benefit as the quality of evidence is very low. Methotrexate may not be of benefit and IFN beta-1a is probably not of benefit.We need further research to identify predictors of response to different treatments and to compare their long-term benefits, safety and cost-effectiveness. There is a need for more randomised trials of immunosuppressive and immunomodulatory agents, routes of administration, and treatments for symptoms of CIDP.

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Conflict of interest statement

ALO receives research grant support from the U.S. National Institutes of Health and Department of Defence; she has no commercial interests.

CHC is a co‐author of two papers cited in this overview: a study of plasma exchange in CIDP (Hahn 1996), and a cost‐benefit analysis of IVIg in CIDP (Blackhouse 2010).

RACH holds or has held consultancies with companies which manufacture human immunoglobulin: Baxter, CSL Behring, Grifols, LFB, and Octapharma, and with Novartis, which is undertaking a trial of fingolimod in CIDP. RACH is co‐author of three CSRs Hughes 2015 (steroids), Mehndiratta 2015 (plasma exchange), Mahdi‐Rogers 2013 (other immunomodulatory treatment) incorporated in this overview. RACH was co‐ordinator, or steering committee chair, and author of the following trials included in the overview: Hadden 1999, Hughes 2001, Hughes 2010, Hughes 2013, RMC 2009, and Thompson 1996. He is Honorary Member of the Board of GBS CIDP Foundation International and Medical Patron of GBS Support Group UK now called 'gain'. He is a member of the Cochrane Neuromuscular Editorial Board but played no role in the editorial process for this overview.

MPTL was co‐author of one of the CSRs included in this overview (Stork 2015). He has received honoraria for consultation from Baxter Pharmaceuticals, CSL Behring and UCB Pharma and travel support grant from Grifols and Baxalta (previously Baxter Phamaceuticals), all manufacturers of IVIG. He was a blinded investigator in the study of Comi et al. 2002. MPTL is a blinded investigator in the CSL Behring PATH studies (ongoing). and was a blinded investigator in the FORCIDP study, closed in 2016 for futility. He is Co‐ordinating Editor of Cochrane Neuromuscular but played no role in the editorial process for this overview.

CF holds a consultancy with CSL Behring (for which his institution receives payment). He was formerly the group Statistician in Cochrane Neuromuscular. He played no part in the editorial process of this overview.

IvS was chief investigator of one of the included trials (Van Schaik 2010) and co‐author of one of the CSRs included in this overview (Eftimov 2013). He chairs a steering committee for CSL‐Behring and received departmental honoraria for serving on scientific advisory boards for CSL‐Behring, Baxalta, and UCB. He received speakers fees from CSL‐Behring and Kedrion. Departmental research support has be granted by The Netherlands Organisation for Scientific Research, and the Dutch Prinses Beatrix Spierfonds. All lecturing and consulting fees for INS were donated to the Stichting Klinische Neurologie, a local foundation that supports research in the field of neurological disorders. He serves on the editorial board of the Cochrane Neuromuscular Disease Group, is a member of the organising committee of the Inflammatory Neuropathy Consortium (INC), a standing committee of the Peripheral Nerve Society and 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).

He played no part in the editorial process for this overview.

Figures

1
1
Study flow diagram. We are unable to calculate numbers of papers following deduplication and numbers reviewed in full text, as different authors reviewed several searches during development of the overview.
See this image and copyright information in PMC

Update of

  • doi: 10.1002/14651858.CD010369

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References to other published versions of this review

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