Chronic Inflammatory Demyelinating Polyneuropathy (CIDP): Current Therapies and Future Approaches

Abstract

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired immune-mediated polyradiculoneuropathy leading to disability via inflammatory demyelination of peripheral nerves. Various therapeutic approaches with different mechanisms of action are established for the treatment of CIDP. Of those, corticosteroids, intravenous or subcutaneous immunoglobulin, or plasma exchange are established first-line therapies as suggested by the recently revised EAN/PNS guidelines for the management of CIDP. In special cases, immunosuppressants or rituximab may be used. Novel therapeutic approaches currently undergoing clinical studies include molecules or monoclonal antibodies interacting with Fc receptors on immune cells to alleviate immune-mediated neuronal damage. Despite various established therapies and the current development of novel therapeutics, treatment of CIDP remains challenging due to an inter-individually heterogeneous disease course and the lack of surrogate parameters to predict the risk of clinical deterioration.

Immunomodulatory effects of intravenous and subcutaneous immunoglobulin in chronic inflammatory demyelinating polyneuropathy: An observational study

“…However, as immunoglobulin effects are manifold beyond the DC-SIGN pathway [7], and inter-cohort cellular and clinical parameters were comparable, we did not find evidence for any therapeutical the anti-inflammatory potential of IL-33 in immune neuropathies has been described [37], it appears controversial that serum levels of MIP-1α positively correlated with this anti-inflammatory cytokine. MIP-1α is a macrophage-attracting factor that is increased in CIDP, and its nerve tissue levels correlated with neuroinflammation in experimental autoimmune neuritis [38,39].…”

“…Additionally, immunoglobulin pre‐treatment and, in some patients, secondary axonal damage and/or a placebo effect, might pose confounders that could mask possible interactions between cytokines and immune cells in these patients. However, as immunoglobulin effects are manifold beyond the DC‐SIGN pathway [7], and inter‐cohort cellular and clinical parameters were comparable, we did not find evidence for any therapeutical relevance of the differential IL‐4/IL‐33/CD32b/c + findings between the CIDP cohorts. A correlation of IL‐33 and MIP‐1α persisted 7 days after IVIg or SCIg treatment in the two CIDP cohorts, which could generally suggest an immunomodulatory pathway.…”

“…Intravenous (IVIg) and subcutaneous immunoglobulin (SCIg) are pooled immunoglobulin G (IgG) solutions from more than 1000 healthy donors and are first-line therapies for CIDP [7]. In common variable immunodeficiency (CVID), IVIg or SCIg are used to substitute serum IgG [8,9].…”

Therapeutic progress and future prospects of chronic inflammatory demyelinating polyradiculoneuropathy

“…Chronic inflammatory demyelinating polyneuropathy (CIDP) is a heterogeneous immune-mediated neuropathy characterized by predominant demyelination of the motor and sensory nerves. 1 CIDP is usually indicated by slowly progressive, symmetric, proximal, and distal paresis and sensory dysfunction, which develops over 2 mo, and has a progressive and/or relapsing course. 2 Clinical presentations different from those of typical CIDP are considered CIDP variants, which include distal, multifocal, focal, motor, and sensory CIDP.…”

The gut microbiome in intravenous immunoglobulin‐treated chronic inflammatory demyelinating polyneuropathy

“…Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune disease caused by an aberrant immune response that involves autoantibodies and pro‐inflammatory Th1 and Th17 cells [8]. Intravenous immunoglobulin (IVIg), an IgG‐enriched blood plasma derivate, is a standard treatment for CIDP [9].…”