Antozzi Carlo
Neuroimmunology and Muscle Pathology Unit, National Neurological Institute Foundation Carlo Besta, Via Celoria 11, 20133 Milan, Italy.
Atheroscler Suppl. 2013 Jan;14(1):219-22. doi: 10.1016/j.atherosclerosissup.2012.10.027.
Autoimmune ion channel disorders of the peripheral nervous system include myasthenia gravis, the Lambert-Eaton myasthenic syndrome, acquired neuromyotonia and autoimmune autonomic ganglionopathies. These disorders are characterized by the common feature of being mediated by IgG autoantibodies against identified target antigens, i.e. the acetylcholine receptor, the voltage-gated calcium and potassium channels, and the neuronal acetylcholine receptor. Moreover, experimental animal models have been identified for these diseases that respond to immunotherapy and are improved by plasmapheresis. On this basis, autoimmune ion channel disorders represent the ideal candidate for therapeutic apheresis. Immunoadsorption can be the treatment of choice when intensive apheretic protocols or long-term treatments must be performed, in patients needing frequent apheresis to keep a stable clinical condition, in case of unresponsiveness to corticosteroids and immunosuppressive treatments, or failure with TPE or intravenous immunoglobulins, and in patients with severe contraindications to long-term corticosteroids.
外周神经系统的自身免疫性离子通道疾病包括重症肌无力、兰伯特-伊顿肌无力综合征、获得性神经性肌强直和自身免疫性自主神经节病。这些疾病的共同特征是由针对特定靶抗原的IgG自身抗体介导,即乙酰胆碱受体、电压门控钙通道和钾通道以及神经元乙酰胆碱受体。此外,已经确定了这些疾病的实验动物模型,它们对免疫疗法有反应,血浆置换可改善病情。在此基础上,自身免疫性离子通道疾病是治疗性血液成分单采的理想候选对象。当必须进行强化血液成分单采方案或长期治疗时,在需要频繁进行血液成分单采以维持稳定临床状况的患者中,在对皮质类固醇和免疫抑制治疗无反应的情况下,或在治疗性血浆置换或静脉注射免疫球蛋白失败时,以及在有长期使用皮质类固醇严重禁忌证的患者中,免疫吸附可能是首选治疗方法。