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Case Reports
. 2022 May 11:27:100404.
doi: 10.1016/j.ensci.2022.100404. eCollection 2022 Jun.

High-dose immunoglobulin-dependent chronic demyelinating inflammatory polyneuropathy successfully managed with subcutaneous immunoglobulin using pharmacokinetic analysis

Satomi Hiya  1 , Satoru Fujiwara  1 , Fumiaki Tanaka  2 , Nobuo Kohara  1 , Michi Kawamoto  1
Affiliations
Case Reports

High-dose immunoglobulin-dependent chronic demyelinating inflammatory polyneuropathy successfully managed with subcutaneous immunoglobulin using pharmacokinetic analysis

Satomi Hiya et al. eNeurologicalSci. .

Erratum in

Abstract

Immunoglobulin G therapy for chronic inflammatory demyelinating polyneuropathy (CIDP) often requires individual dose adjustments because of the heterogeneity of pathogenesis and varying catabolic rates. However, currently available pharmacokinetic studies of immunoglobulin G therapy do not consider individual differences. We conducted a pharmacokinetic study of both intravenous immunoglobulin and subcutaneous immunoglobulin in a single patient with CIDP who was dependent on high-dose immunoglobulin treatment. This patient-a 77-year-old man with symmetrical limb weakness, diffuse demyelination determined by a nerve conduction study, and lacking autoantibodies-was treated with intravenous immunoglobulin and experienced severe fluctuations in symptoms. We transitioned him to subcutaneous immunoglobulin: his serum immunoglobulin G levels stabilised and he experienced symptomatic relief. Monitoring of serum immunoglobulin G concentrations revealed volatile changes following intravenous immunoglobulin administration which stabilised following subcutaneous immunoglobulin treatment. This suggests that subcutaneous immunoglobulin is a preferable long-term treatment option, especially for high-dose immunoglobulin-dependent patients with CIDP.

Keywords: Autoantibodies; Chronic inflammatory demyelinating polyradiculoneuropathy; Diffuse demyelination; Immunoglobin G; Intravenous immunoglobulin; Pharmacokinetic analysis; Subcutaneous immunoglobulin.

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Conflict of interest statement

The authors report no conflict of interest.

Figures

Fig. 1
Fig. 1
Treatment, serum IgG level and symptom response. The patient showed IgG dependency. Both deterioration and recovery followed after IgG level decrease and increase with some delay. The patient achieved symptom stability with subcutaneous immunoglobulin use but experienced two less-severe relapses. PSL: prednisolone, CsA: cyclosporin A, MMF: mycophenol mofetil, IVIg: intravenous immunoglobulin, SCIg: subcutaneous immunoglobulin, MRC: medical research council.
Fig. 2
Fig. 2
Simulated change of serum IgG levels.
See this image and copyright information in PMC

References

    1. Van den Bergh P.Y.K., van Doorn P.A., Hadden R.D.M., et al. European academy of neurology/peripheral nerve society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force-second revision. J. Peripher. Nerv. Syst. 2021;26:242–268. - PubMed
    1. Allen J.A., Berger M., Querol L., et al. Individualized immunoglobulin therapy in chronic immune-mediated peripheral neuropathies. J. Peripher. Nerv. Syst. 2018;23:78–87. doi: 10.1111/jns.12262. - DOI - PMC - PubMed
    1. Debs R., Reach P., Cret C., et al. A new treatment regimen with high-dose and fractioned immunoglobulin in a special subgroup of severe and dependent CIDP patients. Int. J. Neurosci. 2017;127:864–872. doi: 10.1080/00207454.2016.1269328. - DOI - PubMed
    1. Doneddu P.E., Cocito D., Manganelli F., et al. Atypical CIDP: diagnostic criteria, progression and treatment response. Data from the Italian CIDP database. J. Neurol. Neurosurg. Psychiatry. 2019;90:125–132. - PubMed
    1. Tortorici M.A., Lawo J.P., Weide R., et al. Privigen® has similar pharmacokinetic properties in primary and secondary immune deficiency. Int. Immunopharmacol. 2019;66:119–126. doi: 10.1136/jnnp-2018-318714. - DOI - PubMed

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