Berta E, Confalonieri P, Simoncini O, Bernardi G, Busnach G, Mantegazza R, Cornelio F, Antozzi C
Neuromuscular Research Center, National Neurological Institute C. Besta, Milano, Italy.
Int J Artif Organs. 1994 Nov;17(11):603-8.
Myasthenia Gravis is an autoimmune disease in which autoantibodies to the acetylcholine receptor interfere with neuromuscular transmission. Plasma exchange is effective in temporarily relieving the symptoms of the disease, but for repeated use the lack of selectivity and need for replacement fluids (which increases the risk of contracting viral diseases) are important drawbacks. Staphylococcal protein A, a potent ligand for immunoglobulins, that interacts negligibly with other plasma proteins, appears to be an optimal candidate for removing antiacetylcholine receptor antibodies, which are mostly IgG. We treated three patients with severe immunosuppression-resistant myasthenia gravis with protein A immunoadsorption. Neurological impairment significantly improved in all patients. After immunoadsorption of 1.5-2 plasma volumes per session, the mean percentage reductions for serum IgG and specific autoantibodies were 71% and 82% respectively. No major side effects occurred. Protein A immunoadsorption appears to be a safe, efficient and effective alternative to plasmaexchange for selected myasthenic patients requiring prolonged apheresis.
重症肌无力是一种自身免疫性疾病，其中针对乙酰胆碱受体的自身抗体干扰神经肌肉传递。血浆置换可有效暂时缓解该疾病的症状，但反复使用时缺乏选择性以及需要补充液体（这增加了感染病毒性疾病的风险）是重要缺点。葡萄球菌蛋白A是一种有效的免疫球蛋白配体，与其他血浆蛋白的相互作用可忽略不计，似乎是去除主要为IgG的抗乙酰胆碱受体抗体的最佳候选物。我们用蛋白A免疫吸附法治疗了3例严重免疫抑制抵抗性重症肌无力患者。所有患者的神经功能障碍均有显著改善。每次免疫吸附1.5 - 2个血浆量后，血清IgG和特异性自身抗体的平均降低百分比分别为71%和82%。未发生重大副作用。对于需要长期进行血液分离术的特定重症肌无力患者，蛋白A免疫吸附似乎是一种安全、高效且有效的血浆置换替代方法。
