Corticosteroids in chronic inflammatory demyelinating polyneuropathy

G G A Van Lieverloo,F Eftimov,E Nobile-Orazio,F Gallia,P E Doneddu,L Wieske,S Peric,C Verhamme,A Nikolic,I Basta,I N Van Schaik

doi:10.1007/s00415-018-8948-y

G G A Van Lieverloo, F Eftimov + Show 9 more

Open Access

https://doi.org/10.1007/s00415-018-8948-y

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Abstract

BackgroundChronic inflammatory demyelinating polyneuropathy (CIDP) can be treated with corticosteroids or intravenous immunoglobulins. Various corticosteroid regimens are currently used in CIDP, but it is unknown whether they are equally efficacious. In this retrospective study, we compared efficacy and safety of three corticosteroid regimens in CIDP patients.MethodsWe included treatment naïve patients that fulfilled the EFNS/PNS criteria for CIDP. Patients were treated with corticosteroids according to the local protocol of three CIDP expertise centres. Corticosteroid regimens consisted of daily oral prednisolone, pulsed oral dexamethasone, or pulsed intravenous methylprednisolone. Outcomes were number of responders to treatment, remission rate of treatment responders, overall probability of 5-year remission, and the occurrence of adverse events.ResultsA total of 125 patients were included. Sixty-seven (54%) patients received daily prednisone or prednisolone, 37 (30%) pulsed dexamethasone, and 21 (17%) pulsed intravenous methylprednisolone. Overall, 60% (95% CI 51–69%) responded to corticosteroids, with no significant difference between the three treatment regimens (p = 0.56). From the 75 responders, 61% (95% CI 50–73%) remained in remission, during a median follow-up of 55 months (range 1–197 months). The probability of responders reaching 5-year remission was 55% (95% Cl 44–70%), with no difference between the three groups. Adverse events leading to a change in treatment occurred in ten patients (8%). Two patients had a serious adverse event.ConclusionCorticosteroids lead to improvement in 60% of patients and to remission in 61% of treatment responders. There were no differences between treatment modalities in terms of efficacy and safety.

Highlights

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated disease of the peripheral nerves that causes sensory and motor impairment
To avoid adverse events associated with corticosteroids, most patients in high-income countries are treated with intravenous immunoglobulin (IVIg), which is associated with less adverse events
Seventy-five CIDP patients (60%, 95% CI 51–69%) were considered responders after corticosteroid treatment; 57% after prednisolone, 68% after dexamethasone, and 57% after methylprednisolone treatment (Table 2)

Summary

Introduction

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated disease of the peripheral nerves that causes sensory and motor impairment. 80% of patients respond well to corticosteroids, intravenous immunoglobulin (IVIg), or plasma exchange [1,2,3]. The decision which treatment to start first in an individual patient is difficult as there are no good predictors of treatment response Both corticosteroids and IVIg have their advantages and drawbacks. Corticosteroids are easy to administer, cheap, Journal of Neurology (2018) 265:2052–2059 and may lead to long-term remission in CIDP more often compared to IVIg [4,5,6,7]. Chronic inflammatory demyelinating polyneuropathy (CIDP) can be treated with corticosteroids or intravenous immunoglobulins. 60% (95% CI 51–69%) responded to corticosteroids, with no significant difference between the three treatment regimens (p = 0.56). Conclusion Corticosteroids lead to improvement in 60% of patients and to remission in 61% of treatment responders. There were no differences between treatment modalities in terms of efficacy and safety

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