Immunoadsorption in patients with autoimmune ion channel disorders of the peripheral nervous system.
Autoimmune ion channel disorders of the peripheral nervous system include myasthenia gravis, the Lambert-Eaton myasthenic syndrome, acquired neuromyotonia and autoimmune autonomic ganglionopathies. These disorders are characterized by the common feature of being mediated by IgG autoantibodies against identified target antigens, i.e. the acetylcholine receptor, the voltage-gated calcium and potassium channels, and the neuronal acetylcholine receptor. Moreover, experimental animal models have been identified for these diseases that respond to immunotherapy and are improved by plasmapheresis. On this basis, autoimmune ion channel disorders represent the ideal candidate for therapeutic apheresis. Immunoadsorption can be the treatment of choice when intensive apheretic protocols or long-term treatments must be performed, in patients needing frequent apheresis to keep a stable clinical condition, in case of unresponsiveness to corticosteroids and immunosuppressive treatments, or failure with TPE or intravenous immunoglobulins, and in patients with severe contraindications to long-term corticosteroids.
Neuroimmunology and Muscle Pathology Unit, National Neurological Institute Foundation Carlo Besta, Via Celoria 11, 20133 Milan, Italy. antozzi@istituto-besta.it
Carlo
C
Antozzi
D000327:Adsorption; D001323:Autoantibodies / Q000097:blood*; D020274:Autoimmune Diseases of the Nervous System / Q000097:blood / Q000175:diagnosis / Q000276:immunology / Q000628:therapy*; D015551:Autoimmunity; D001342:Autonomic Nervous System Diseases / Q000097:blood / Q000276:immunology / Q000628:therapy; D015415:Biomarkers / Q000097:blood; D001781:Blood Component Removal / Q000379:methods*; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D007163:Immunosorbent Techniques; D007164:Immunosorbents / Q000627:therapeutic use*; D007473:Ion Channels / Q000276:immunology*; D020386:Isaacs Syndrome / Q000097:blood / Q000276:immunology / Q000628:therapy; D015624:Lambert-Eaton Myasthenic Syndrome / Q000097:blood / Q000276:immunology / Q000628:therapy; D009157:Myasthenia Gravis / Q000097:blood / Q000276:immunology / Q000628:therapy; D010523:Peripheral Nervous System Diseases / Q000097:blood / Q000175:diagnosis / Q000276:immunology / Q000628:therapy*; D016896:Treatment Outcome
D001323:Autoantibodies; D015415:Biomarkers; D007074:Immunoglobulin G; D007164:Immunosorbents; D007473:Ion Channels