Currently, autoimmune diseases of the peripheral nervous system are mainly treated with exogenous, high-dose intravenous immunoglobulin (IVIg). However, due to the increasing shortage of IVIg and other problems, it is crucial to develop new therapies, one of which is the use of B-cell-depleting monoclonal antibodies. This route has played a prominent role in the treatment of chronic autoimmune demyelinating neuropathies, particularly in anti-MAG antibody neuropathy and autoimmune neuropathies with nodal/paranoid antigen antibodies unresponsive to IVIg.
Chronic inflammatory demyelinating polyneuropathy (CIDP) is a type of inflammatory polyneuropathy characterized by progressive symmetric (lasting more than 2 months) or recurrent remitting sensorimotor deficits. Clinical studies have shown that a patient with CIDP received and responded to rituximab after the failure of IVIg and steroid therapy.
Nodo-paranodopathies are autoimmune neuropathies. Patients with this disease respond poorly to IVIg and antibodies (mainly IgG4 and IgG3 subtypes) to nodal-paranodal antigens (i.e., neurofascial-155, contactin-1, and caspr1 in the region of the paranodal, and neurofascin-186/-140 in the region of the nodal). Clinical studies have shown that this type of disease appears to respond to rituximab.
Neuropathy with anti-MAG antibodies is the most common IgM paraproteinemic neuropathy and is characterized by predominant sensory symptoms, ataxic gait, and upper limb tremors, with motor involvement and disability occurring late in the course of the disease. Clinical studies have demonstrated that the administration of rituximab improves the disability caused by anti-MAG neuropathy. However, there are very few immunotherapies that are effective in treating anti-MAG neuropathy. Thus, it is necessary to develop monoclonal antibodies that are more effective against B-cell depletion.
Multifocal motor neuropathy (MMN) is a rare form of acquired motor neuropathy that primarily affects young men and is characterized by progressive asymmetric weakness without sensory loss.
MMN does not respond to steroids or plasma replacement, and the response to IVIg therapy remains unclear.
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