Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a term for a group of acquired, immune-mediated inflammatory demyelinating disorders of the peripheral nervous system. Most patients with CIDP respond to first-line therapy with intravenous immunoglobulin (IVIG), plasmapheresis, and/or corticosteroids. Conventional immunosuppressive drugs are of no proven benefit. Biological agents directed at key aspects of the CIDP immunopathogenic pathway have gained increasing attention due to the unpredictable efficacy and overall health risks of non-targeted immunosuppressive drugs. Presently, there exists insufficient clinical experience with biological therapy to allow specific treatment recommendations for CIDP. The challenge remains to identify drug-naïve or treatment-resistant CIDP patients who will most likely respond to targeted immunotherapy.
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