Chronic inflammatory demyelinating polyradiculoneuropathy: from pathology to phenotype

Abstract

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an inflammatory neuropathy, classically characterised by a slowly progressive onset and symmetrical, sensorimotor involvement. However, there are many phenotypic variants, suggesting that CIDP may not be a discrete disease entity but rather a spectrum of related conditions. While the abiding theory of CIDP pathogenesis is that cell-mediated and humoral mechanisms act together in an aberrant immune response to cause damage to peripheral nerves, the relative contributions of T cell and autoantibody responses remain largely undefined. In animal models of spontaneous inflammatory neuropathy, T cell responses to defined myelin antigens are responsible. In other human inflammatory neuropathies, there is evidence of antibody responses to Schwann cell, compact myelin or nodal antigens. In this review, the roles of the cellular and humoral immune systems in the pathogenesis of CIDP will be discussed. In time, it is anticipated that delineation of clinical phenotypes and the underlying disease mechanisms might help guide diagnostic and individualised treatment strategies for CIDP.

Corneal confocal microscopy in chronic inflammatory demyelinating polyneuropathy

“…Immune‐mediated disorders of the peripheral nervous system (PNS) exhibit a wide variety of clinical presentations and can be challenging in their diagnosis and treatment 1, 2. Despite established criteria to diagnose chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), there is significant clinical heterogeneity in relation to clinical course and response to treatment 3.…”

Immunotherapy in Peripheral Neuropathies

“…The main issue for immunotherapy is the great variability in clinical presentation and course of these diseases, together with the different responses to treatment according to the clinical phenotypes. In the same vein, new subsets have been recently reported in the spectrum of CIDP, which may lead to different treatment options [7,8].…”

Chronic Inflammatory Demyelinating Polyradiculoneuropathy 101—Pitfalls and Pearls of Diagnosis and Treatment

“…6 There are many phenotypic variants of CIDP (Table 1), which suggests that the disorder may not be a discrete entity, but a spectrum of conditions. 7 Elevated levels of cerebrospinal fluid (CSF) protein are present in the majority of patients although normal CSF results do not exclude the diagnosis of CIDP. 7,8 Currently, there are no well-established biomarkers, although autoantibodies to contactin-1 and neurofascin-155 define CIDP subsets of patients with specific clinical features.…”