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. 2023 Mar 10;15(1):405-414.
doi: 10.3390/neurolint15010027.

Parameters Associated with the Required Drug Dose of Intravenous Immunoglobulin in Stable Chronic Inflammatory Demyelinating Polyradiculoneuropathy

Ludger Feyen  1   2   3 , Christina Schaub  4 , Julian Zimmermann  4 , Louisa Nitsch  4
Affiliations

Parameters Associated with the Required Drug Dose of Intravenous Immunoglobulin in Stable Chronic Inflammatory Demyelinating Polyradiculoneuropathy

Ludger Feyen et al. Neurol Int. .

Abstract

Background: Intravenous immunoglobulin (IVIg) is efficient and one of very few treatment options for patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). However, finding the optimal dose of IVIg for individual CIDP patients remains challenging. The dose of IVIg needs to be adjusted individually. Considering the high healthcare costs of IVIg therapy, the overtreatment of some patients seen in placebo studies and the shortage of IVIg we recently experienced, as well as identifying factors associated with the required dose of IVIg in maintenance treatment, is extremely important. Thus, in this retrospective study, we analyze characteristics of patients with stable CIDP, which are associated with the required drug dose.

Methods: 32 patients with stable CIDP treated with IVIg between July 2021 and July 2022 were identified from our database and included in this retrospective study. Patients' characteristics were registered, and parameters were identified that were associated with the IVIg dose.

Results: Age, cerebrospinal fluid protein elevation, disease duration, delay between symptom onset/diagnosis, Inflammatory Neuropathy Cause and Treatment (INCAT) score, and Medical Research Council Sum Score (MRC SS) were significantly associated with the required drug dose. In addition, an association of age, sex, elevated CSF protein, time interval between symptom onset and diagnosis, and the MRC SS with the required IVIg dose could be demonstrated in the multivariable regression analysis.

Conclusions: Our model, which is based on routine parameters that are simple to address in the clinical practice, can be useful in adjusting the IVIg dose in patients with stable CIDP.

Keywords: CIDP; IVIg; dosing; maintenance therapy.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Figure 1
Figure 1
Mechanisms of action of intravenous immunoglobulins (IVIg) in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). It is hypothesized that the therapeutic effect of IVIg in CIDP is mediated by multiple mechanisms. IVIg treatment inhibits autoantibodies to bind to their antigen (anti-idiotypic effect of IVIg). The saturation of the neonatal Fc receptor (FcRn) leads to reduction of the circulating antibodies. In addition, IVIg seems to have a direct inhibitory effect on macrophage activation and interferes with activation of co-stimulatory molecules and cell-adhesion molecules. IVIg therapy leads to inhibition of complement activity, which is important for antibody-mediated cytotoxicity and macrophage activation.
Figure 2
Figure 2
Individual patients’ characteristics. An overview of the characteristics of the CIDP patients is presented. Abbreviations: MGUS monoclonal gammopathy of undetermined significance, GBS Guillain–Barre Syndrome, CSF Cerebrospinal fluid.
Figure 3
Figure 3
Box plot diagram for CSF protein elevation and sex. For the two characteristics (CSF protein elevation and sex) with the highest coefficients in the multivariabe regression model, a box plot diagram depicting the pattern of data distribution is shown.
See this image and copyright information in PMC

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