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Review
. 2021 Mar 22;18(1):78.
doi: 10.1186/s12974-021-02113-2.

The immune response and aging in chronic inflammatory demyelinating polyradiculoneuropathy

Affiliations
Review

The immune response and aging in chronic inflammatory demyelinating polyradiculoneuropathy

Kathleen M Hagen et al. J Neuroinflammation. .

Abstract

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) consists of various autoimmune subtypes in which the peripheral nervous system (PNS) is attacked. CIDP can follow a relapsing-remitting or progressive course where the resultant demyelination caused by immune cells (e.g., T cells, macrophages) and antibodies can lead to disability in patients. Importantly, the age of CIDP patients has a role in their symptomology and specific variants have been associated with differing ages of onset. Furthermore, older patients have a decreased frequency of functional recovery after CIDP insult. This may be related to perturbations in immune cell populations that could exacerbate the disease with increasing age. In the present review, the immune profile of typical CIDP will be discussed followed by inferences into the potential role of relevant aging immune cell populations. Atypical variants will also be briefly reviewed followed by an examination of the available studies on the immunology underlying them.

Keywords: Aging; Chronic inflammatory demyelinating polyradiculoneuropathy; Immune system; Immunosenescence; Peripheral nervous system.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Proposed mechanism of age and immunological contributions to typical CIDP pathology. In CIDP, there is a decline in regulatory T cells which are opposite to the age-related increase in these cells. In combination with a decline in naïve T cells with age, this may contribute to the CIDP-related increase in Th17 cells, thus creating an imbalance in Tregs and Th17 cells. Due to dysregulation of the immune system, this may allow for further pathological contributions of the infiltrating natural killer T cells and distorted CD8+ T cell repertoire seen in CIDP. Also, with age, there is an increase in macrophages within the peripheral nerve and in combination with an increase in macrophages due to CIDP, and this could contribute to an age-related increase in disease severity. Orange arrows = age-related contribution; green arrows = disease-related contribution; combination of green and orange arrows = cumulative contribution of age and disease

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