Wagner S, Janzen R W, Mohs C, Pohlmann S, Klingel R, Grützmacher P W
Medizinische Klinik II, Schwerpunkt für Nephrologie, Hochdruck und Gefässkrankheiten, Markuskrankenhaus, Frankfurt/Main.
Dtsch Med Wochenschr. 2008 Nov;133(46):2377-82. doi: 10.1055/s-0028-1100928. Epub 2008 Nov 4.
Myasthenia gravis in the majority of patients is a well treatable neurological autoimmune disorder with a prevalence of 60-150 per million. For the treatment of myasthenic crisis in the intensive care unit the use of therapeutic apheresis, e. g. immunoadsorption or plasma exchange, is well established due to its rapid therapeutic effect, whereas the necessity in long term treatment is still questioned. Aim of this retrospective cohort-study was the assessment of patients with refractory myasthenia gravis in Germany treated by regular immunoadsorption, the characterization of previous therapies and the efficacy of long-term treatment.
In total 14 patients (9 women, 5 men, mean age: 40.5 years) were identified in Germany using regular therapeutic apheresis. 13 were treated with different modes of immunoadsorption (10 yen l-tryptophan-adsorption, 2 yen epitope-specific adsorption, 1 yen polyclonal sheep antibody on sepharose) and 1 with plasma exchange. Mean duration of standard treatment of myasthenia gravis before initiation of regular apheresis was 7.8 years.
Average duration of analyzed apheresis treatment was 6.4 years, with a mean treatment-interval of 1.1 per week. Mean reduction rate of autoantibodies against acetylcholine-receptor-protein was 50-60 % per session. After initiation of immunoadsorption the mean time of hospitalisation decreased significantly by app. 60 %. In particular the number of myasthenic crises could be reduced by 89 % per year. Tolerability of immunoadsorption was very good, no severe adverse events occurred.
In conclusion, for the treatment of the subgroup of myasthenia gravis patients becoming refractory to standard treatment immunoadsorption should be regarded as integral part of the therapeutic armamentarium to stabilize and optimize the state of neurologic rehabilitation. This evaluation should be also carefully considered by carriers of health care cost as currently best available evidence to decide on appropriate treatment regimens for these rare patients.
重症肌无力在大多数患者中是一种可有效治疗的神经自身免疫性疾病，每百万人口中的患病率为60 - 150例。在重症监护病房治疗重症肌无力危象时，治疗性血液成分单采术（如免疫吸附或血浆置换）因其快速的治疗效果而得到广泛应用，然而其在长期治疗中的必要性仍受到质疑。这项回顾性队列研究的目的是评估德国接受常规免疫吸附治疗的难治性重症肌无力患者，描述既往治疗情况以及长期治疗的疗效。
在德国，共确定了14例患者（9名女性，5名男性，平均年龄：40.5岁）接受常规治疗性血液成分单采术。13例接受了不同模式的免疫吸附治疗（10例采用L - 色氨酸吸附，2例采用表位特异性吸附，1例采用琼脂糖上的多克隆羊抗体），1例接受血浆置换。在开始常规血液成分单采术之前，重症肌无力标准治疗的平均持续时间为7.8年。
分析的血液成分单采术治疗的平均持续时间为6.4年，平均治疗间隔为每周1.1次。每次治疗后，抗乙酰胆碱受体蛋白自身抗体的平均降低率为50 - 60%。开始免疫吸附治疗后，平均住院时间显著减少约60%。特别是每年重症肌无力危象的数量可减少89%。免疫吸附的耐受性非常好，未发生严重不良事件。
总之，对于对标准治疗难治的重症肌无力患者亚组，免疫吸附应被视为治疗手段的一个组成部分，以稳定和优化神经康复状态。医疗费用承担者也应仔细考虑这一评估结果，因为这是目前关于为这些罕见患者确定合适治疗方案的最佳可用证据。
