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Review
. 2019 Feb 12:7:3.
doi: 10.1186/s40364-019-0154-2. eCollection 2019.

Measuring disease activity and predicting response to intravenous immunoglobulin in chronic inflammatory demyelinating polyneuropathy

Anthony Khoo  1   2 , Joseph Frasca  1   2 , David Schultz  1   2
Affiliations
Review

Measuring disease activity and predicting response to intravenous immunoglobulin in chronic inflammatory demyelinating polyneuropathy

Anthony Khoo et al. Biomark Res. .

Abstract

Chronic inflammatory demyelinating polyneuropathy (CIDP) is characterised by significant clinical heterogeneity and as such reliable biomarkers are required to measure disease activity and assess treatment response. Recent advances in our understanding of disease pathogenesis and the discovery of novel serum-based, electrophysiologic and imaging biomarkers allow clinicians to make more informed decisions regarding individualised treatment regimes. As a chronic immune-mediated process typified by relapse following withdrawal of immunomodulatory therapy, a substantial proportion of patients with CIDP require long term treatment with intravenous immunoglobulin (IVIg), a scarce and expensive donor-derived resource. The required duration and intensity of immunoglobulin treatment vary widely between individuals, highlighting both the heterogeneous nature of the underlying disease process as well as the variable pharmacologic properties of IVIg. This review outlines the use of multimodal biomarkers in the longitudinal evaluation of nerve injury and how recent developments have impacted our ability to predict both response to immunoglobulin administration and its withdrawal.

Keywords: Biomarkers; Chronic inflammatory demyelinating polyneuropathy; Intravenous immunoglobulin; Magnetic resonance neurography; Nerve ultrasound; Paranodal antibodies.

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Conflict of interest statement

Not applicable.Not applicable.The authors declare that they have no competing interests.Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

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