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Short-term and long-term effects of immunoadsorption in refractory chronic inflammatory demyelinating polyneuropathy: a prospective study in 17 patients

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Abstract

Objective

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare, autoimmune-inflammatory disease of the peripheral nervous system. Recently, various immunoglobulin G4 (IgG4) type auto-antibodies have been described in patients with CIDP which can effectively be removed by immunoadsorption (IA). Therefore, we prospectively evaluated the therapeutic effect of IA in 17 patients with progressive CIDP not responding to other treatment.

Methods

We prospectively evaluated the course of disease of 17 patients with CIDP who had insufficiently responded to steroids and/or IVIg previously and who received at least one cycle of IA. As clinical outcome parameter, we used a combined CIDP score of three validated scales comprising disability, motor score, and sensitivity. Seven patients received repeated treatments in regular intervals over a prolonged period up to 46 months.

Results

We observed a small, but significant improvement of the overall score after 2 weeks that mainly reflected an improvement of muscle strength. The median value of the combined CIDP score was 308.0 (266.0–374.5) points before IA and 330.0 (290.0–393.5) points 2 weeks after IA (p = 0.019). More importantly, all but one of seven progressive patients who received long-term immunoadsorption in regular intervals stabilized almost completely. Before IA, these patients lost 6.7 (3.0–13.1) points of combined CIDP score per month. During IA, they lost − 0.1 (0.0–0.8) points per month (p < 0.0001).

Interpretation

Our results suggest that IA might constitute a promising and well-tolerated therapeutic alternative in CIDP for short-term and long-term treatment. We showed that long-term treatment with IA in regular intervals can stabilize the course of disease at least in a subgroup of patients.

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Author notes

  1. Makbule Senel and Hayrettin Tumani have contributed equally.

Authors and Affiliations

  1. Universitätsklinik Ulm, RKU, Oberer Eselsberg 45, 89081, Ulm, Germany

    Johannes Dorst, Albert C. Ludolph, Makbule Senel & Hayrettin Tumani

Authors
  1. Johannes Dorst
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  2. Albert C. Ludolph
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  3. Makbule Senel
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  4. Hayrettin Tumani
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Contributions

JD did the scientific literature search, data collection, and analysis of data. JD, HT, and MS were involved in study conception and design. All authors participated in data interpretation. JD wrote the first draft of the manuscript, and all authors reviewed and critiqued the manuscript. HT and MS contributed equally to this work.

Corresponding author

Correspondence to Johannes Dorst.

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Conflicts of interest

JD reports personal fees from Fresenius Medical Care GmbH and Fresenius Medical Care Deutschland GmbH, outside the submitted work. JD and HT report research and sponsoring grants from Fresenius Medical Care GmbH and Fresenius Medical Care Deutschland GmbH, outside the submitted work. MS has received honoraria for speaking and/or travel grants from Bayer, Biogen, and TEVA, and research funding from the Hertha-Nathorff-Program and University of Ulm; none related to this study. All other authors declare that they have no conflict of interest.

Ethical standards

The study has been approved by the local ethics committee and has been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments. All patients were extensively informed about the procedure, its risks, and possible therapeutic alternatives, and gave their written informed consent.

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Dorst, J., Ludolph, A.C., Senel, M. et al. Short-term and long-term effects of immunoadsorption in refractory chronic inflammatory demyelinating polyneuropathy: a prospective study in 17 patients. J Neurol 265, 2906–2915 (2018). https://doi.org/10.1007/s00415-018-9082-6

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  • DOI: https://doi.org/10.1007/s00415-018-9082-6

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