Gajdos P
Service de Réanimation Médicale, Hôpital Raymond-Poincaré, 104, boulevard Raymond-Poincaré, 92380 Garches.
Ann Med Interne (Paris). 2000 May;151 Suppl 1:1S30-4.
Myasthenia Gravis (MG) is an autoimmune disease characterized by the production of auto antibodies directed against the acetylcholine receptor of the neuro-muscular synapse. The signs and symptoms are a muscular deficit involving the spinal or cranial muscles. The degree of weakness changes spontaneously over shorter or longer periods. Some exacerbations, called myasthenia crisis, involve the respiratory muscle and are life-threatening. The prognosis of these crisis has been transformed by the use of mechanical ventilation. Treatments directed on immune-regulation such as thymectomy, corticosteroids or immunossupressive drugs contribute to the improvement in functional status and reduce the risk of exacerbation. Plasma exchanges lead to a rapid improvement of weakness during exacerbations. They are, despite the lack of controlled study, the reference treatment for acute exacerbations. More recently, high doses of immunoglobulins have been proposed and a controlled study has shown that they could be an alternative for the treatment of acute exacerbations of MG. Indication of immunoglobulin in the long term management of MG is not established.
重症肌无力(MG)是一种自身免疫性疾病,其特征是产生针对神经肌肉突触乙酰胆碱受体的自身抗体。体征和症状为涉及脊髓或颅部肌肉的肌肉功能缺陷。肌无力程度在短时间或长时间内会自发变化。一些加重情况,称为重症肌无力危象,累及呼吸肌,可危及生命。机械通气的应用改变了这些危象的预后。针对免疫调节的治疗,如胸腺切除术、皮质类固醇或免疫抑制药物,有助于改善功能状态并降低加重风险。血浆置换可在病情加重期间迅速改善肌无力症状。尽管缺乏对照研究,但它们仍是急性加重期的参考治疗方法。最近,有人提出使用高剂量免疫球蛋白,一项对照研究表明,它们可作为治疗MG急性加重的替代方法。免疫球蛋白在MG长期管理中的应用指征尚未确立。