CIDP Advanced Immunotherapy

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune-mediated polyneuropathy with progressive or relapsing weakness, sensory loss, and areflexia evolving over more than 8 weeks. Pathogenesis involves T-cell and antibody-mediated attack on Schwann cells and nodal/paranodal proteins. Distinguishing classical demyelinating CIDP from autoimmune nodopathies (anti-NF155, anti-NF186, anti-CNTN1, anti-Caspr1) is critical for therapy choice.

First-line therapies are intravenous immunoglobulin (IVIG), corticosteroids, and plasma exchange. Subcutaneous immunoglobulin (SCIG) provides similar efficacy with home administration and stable trough levels. Refractory or steroid-dependent patients escalate to rituximab (especially in IgG4 nodopathies), cyclophosphamide, mycophenolate, azathioprine, eculizumab in selected cases, and the FcRn inhibitor efgartigimod which has shown promising results.

Treatment goals are sustained remission, minimization of corticosteroid burden, and disability reversal. Functional assessment with INCAT, ONLS, MRC sum score, and grip strength guides response. Treatment failure is reassessed by re-examination of diagnosis, antibody testing for autoimmune nodopathy, and consideration of combination therapy. CIDP atypical variants (DADS, MADSAM Lewis-Sumner, focal, sensory, motor) may respond differently.