Gilhus Nils Erik, Owe Jone F, Hoff Jana Midelfart, Romi Fredrik, Skeie Geir Olve, Aarli Johan A
Department of Clinical Medicine, University of Bergen, 5020 Bergen, Norway.
Autoimmune Dis. 2011;2011:847393. doi: 10.4061/2011/847393. Epub 2011 Oct 5.
Patients with autoimmune myasthenia gravis (MG) should be further classified before initiating therapy, as treatment response varies for ocular versus generalised, early onset versus late onset, and acetylcholine receptor antibody positive versus MuSK antibody positive disease. Most patients need immunosuppression in addition to symptomatic therapy. Prednisolone and azathioprine represent first choice drugs, whereas several second choice options are recommended and should be considered. Thymectomy should be undertaken in MG with thymoma and in generalised, early-onset MG. For MG crises and other acute exacerbations, intravenous immunoglobulin (IvIg) and plasma exchange are equally effective and safe treatments. Children and females in child bearing age need special attention regarding potential side effects of immunosuppressive therapy. MG pathogenesis is known in detail, but the immune therapy is still surprisingly unspecific, without a pin-pointed attack on the defined disease-inducing antigen-antibody reaction being available.
自身免疫性重症肌无力(MG)患者在开始治疗前应进一步分类,因为眼部与全身型、早发型与晚发型、乙酰胆碱受体抗体阳性与肌肉特异性激酶(MuSK)抗体阳性疾病的治疗反应有所不同。大多数患者除了对症治疗外还需要免疫抑制治疗。泼尼松龙和硫唑嘌呤是首选药物,同时推荐并应考虑几种二线选择。患有胸腺瘤的MG患者以及全身型、早发型MG患者应进行胸腺切除术。对于MG危象和其他急性加重情况,静脉注射免疫球蛋白(IvIg)和血浆置换是同样有效且安全的治疗方法。儿童和育龄期女性需要特别关注免疫抑制治疗的潜在副作用。MG的发病机制已得到详细了解,但免疫治疗仍然出人意料地缺乏特异性,目前尚无针对明确的致病抗原 - 抗体反应进行精准攻击的方法。