Neurológia pre prax 6/2011

Therapeutic plasma exchange in the treatment of autoimmune neuromuscular disordes

The aim of therapeutic plasma exchange (TPE) is to remove pathological (autoantibodies, monoclonal proteins, toxins bound to plasma proteins) or increased physiological plasma components (cytokins, kinins). Among neurological diseases, TPE is the accepted first-line therapy (according to American Society for Apheresis [ASFA] – category I) in PANDAS („Pediatric Autoimmune Neuropsychiatric Disorders Associated with streptoccal infections and Sydenham’s chorea“), and the second-line therapy (category II) in acute disseminated encephalomyelitis, multiple sclerosis, Devic disease and some paraneoplastic neurological syndromes with autoantibodies. Autoimmune neuromuscular disorders, however, are the most frequent indications for TPE in neurology: myasthenia gravis (MG), Guillain- Barré syndrome (GBS), chronic inflammatory demyelinating polyneuropathy (CIDP) and paraproteinaemic polyneuropathy IgG/IgA are classified as category I (acc. to ASFA), and Lambert-Eaton myastenic syndrome (LEMS) and acquired neuromyotonia as category II. Guidelines of the European Federation of Neurology, the American Academy of Neurology and the Czech Neurological Society, however, consider TPE as the first-line therapy in GBS and MG only (as the alternative method to intravenous humane immunoglobulin [IVIG] that has more favourable safety profile), while TPF is recommended as the second-line therapy in CIDP, and the evidence of the TPE effect in paraproteinaemic polyneuropathies and LEMS is lacking.

Keywords: plazma exchange, autoantibodies, myasthenia gravis, Guillain-Barré syndrome.