重症筋無力症のアフェレシス治療(<特集>免疫性神経疾患とアフェレシス,新しい展望)
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概要
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Myasthenia gravis (MG) is characterized by easy fatigability and weakness. MG research of the past thirty years showed evidence of neuromuscular blockade by anti-acetylcholine receptor (AChR) antibodies and established a treatment algorithm based in the pathomechanism. Plasma exchange has been a choice of treatment for MG since as early as 1976. Although a randomized controlled trial has not been done, the effect of plasmapheresis including plasma exchange has been documented through many open retrospective or prospective studies that reported a beneficial effect in more than two thirds of patients treated by plasmapheresis. To avoid viral infection by a transfusion of a large volume of albumin or globulin fluids in a simple plasma exchange, double filtration plasmapheresis (DFPP) or immunoadsorption plasmapheresis using Immunosorba TR-350 was widely used. The efficacy of DFPP and immunoadsorption plasmapheresis are similar. The recent purpose of plasmapheresis is treatment of myasthenic crisis, induction therapy for severe/intractable patients, or prevention of worsening after thymectomy. The combination therapy with steroids or immunosuppressive agents should be needed to prevent a rebound phenomenon, for the antibody production after plasmapheresis by negative feedback. The effect of plasmapheresis for MG patients with anti-MuSK antibody is unique. Plasma exchange or DFPP is more effective than immunoadsorption plasmapheresis. Plasmapheresis seems not to influence the long-term prognosis of patients receiving thymectomy and immunosuppressive therapy. Cost and efficacy of plasmapheresis and high-dose intravenous immunoglobulin (IVIg) are similar but more adverse effects have been reported for plasmapheresis than for IVIg. Therefore, the use of plasamapheresis may be limited for therapy of MG. The combination of plasmapheresis and IVlg may be the most powerful induction therapy before immunosuppressive therapy for severe patients.
- 2004-10-31
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