Treatment of CIDP patients with antibodies against paranodal proteins

AbstractChronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired immune ‐mediated polyneuropathy characterized by relapsing or steadily progressive clinical course. A major feature of the syndrome is that it encompasses several pathogeneses, and it is actually assumed that both cellular and humoral immunity can be involved with superiority depending on the particular subtype. While cellular immunity typified by macrophage‐derived demyelination can play an essential role in classical CIDP, other pathogeneses that involve humoral immunity, such as autoantibodies targeting Node of Ranvier proteins (neurofascin155: NF155; contactin1: CNTN1; contactin‐associated protein1: Caspr1; and neurofascin186/140: NF186/140), can bring about functional demyelination and have been discovered in about 5%–15% of CIDP patients. Interestingly, such antibodies commonly belong to the IgG4 subclass, and thus lack complement‐binding sites and compete with other IgG subclas ses for the antigens. Patients with these IgG4 autoantibodies share clinical presentations to a certain extent, and anti‐NF155 and anti‐CNTN1 antibodies are representative examples. Patients with these IgG4 subclass antibodies fulfill the conservative diagnostic criteria for CIDP: distal‐domin ant weakness, tremor, and sensory ataxia. These patients also show poor responses to conventional first‐line therapeutics, and are non‐responders to intravenous immunoglobulins (IVIg) in particular. ...

Source: Clinical and Experimental Neuroimmunology - April 16, 2020 Category: Neurology Authors: Masahiro Iijima Tags: REVIEW ARTICLE (INVITED) Source Type: research