重症肌无力的死亡率和生存率：一项基于丹麦人群的研究。
Mortality and survival in myasthenia gravis: a Danish population based study.
作者信息
Christensen P B, Jensen T S, Tsiropoulos I, Sørensen T, Kjaer M, Højer-Pedersen E, Rasmussen M J, Lehfeldt E
机构信息
Department of Neurology, Aarhus University Hospital, Denmark.
出版信息
J Neurol Neurosurg Psychiatry. 1998 Jan;64(1):78-83. doi: 10.1136/jnnp.64.1.78.
OBJECTIVES
To study mortality and survival of patients with myasthenia gravis.
METHODS
290 patients with myasthenia gravis were studied, including 212 incident cases identified during a comprehensive epidemiological study of myasthenia gravis in western Denmark 1975-89. Follow up was performed on 31 December 1994. Survival curves were constructed using the life table method. Patient data were compared with data from the public Danish population statistics. Death certificates were provided from the National Registry of Death.
RESULTS
The annual average crude mortality rate was 1.8 per million (range 1.5-2.2). The myasthenia gravis related mortality rate (myasthenia gravis as underlying or contributory cause) was 1.4 per million (range 1.1-1.8). The age specific mortality rates were low below 50 years. After this age the mortality increased with age in both sexes; after 60 years more rapidly in men than in women. The overall survival rates three, five, 10, and 20 years from diagnosis were 85%, 81%, 69%, and 63% respectively. The survival of both sexes was shorter than that of the corresponding Danish population. Old age at diagnosis, a classification in Osserman-Genkins group IIB or III, and the presence of a thymoma were associated with a less favourable prognosis. The three, five, 10, and 20 year survival rates of thymectomised patients were 94%, 94%, 86%, and 79% respectively. The corresponding figures for the non-thymectomised patients were 78%, 71%, 56%, and 51%. A Cox regression analysis showed that this apparently significant effect of thymectomy was because the thymectomised patients were younger than the non-thymectomised patients. Furthermore, at the time of diagnosis of myasthenia gravis the non-thymectomised patients had a higher frequency of serious conditions associated with myasthenia gravis than the thymectomised patients.
CONCLUSION
Patients with myasthenia gravis generally have a relative good prognosis although their survival is shorter than that of the corresponding population. Old age, a classification in Osserman-Genkins group III, and the presence of a thymoma are associated with a less favourable prognosis. In this study, the apparently significant effect of thymectomy was because the thymectomised patients were younger than non-thymectomised patients and because the non-thymctomised patients had a higher frequency of serious conditions associated with myasthenia gravis.
目的
研究重症肌无力患者的死亡率和生存率。
方法
对290例重症肌无力患者进行了研究，其中包括在1975 - 1989年丹麦西部重症肌无力综合流行病学研究中确诊的212例新发病例。随访截至1994年12月31日。采用生命表法构建生存曲线。将患者数据与丹麦公共人口统计数据进行比较。死亡证明由国家死亡登记处提供。
结果
年平均粗死亡率为每百万1.8例（范围为1.5 - 2.2）。重症肌无力相关死亡率（以重症肌无力为根本或促成原因）为每百万1.4例（范围为1.1 - 1.8）。50岁以下的年龄别死亡率较低。此后，两性的死亡率均随年龄增加；60岁以后男性的死亡率增长速度快于女性。从诊断起3年、5年、10年和20年的总生存率分别为85%、81%、69%和63%。两性的生存率均低于相应的丹麦人群。诊断时年龄较大、Osserman - Genkins分类为IIB或III级以及存在胸腺瘤与预后较差相关。接受胸腺切除术患者的3年、5年、10年和20年生存率分别为94%、94%、86%和79%。未接受胸腺切除术患者的相应数字为78%、71%、56%和51%。Cox回归分析表明，胸腺切除术的这种明显显著效果是因为接受胸腺切除术的患者比未接受胸腺切除术的患者年轻。此外，在重症肌无力诊断时，未接受胸腺切除术的患者与重症肌无力相关的严重疾病发生率高于接受胸腺切除术的患者。
结论
重症肌无力患者总体预后相对较好，尽管其生存率低于相应人群。年龄较大、Osserman - Genkins分类为III级以及存在胸腺瘤与预后较差相关。在本研究中，胸腺切除术的明显显著效果是因为接受胸腺切除术的患者比未接受胸腺切除术的患者年轻，且未接受胸腺切除术的患者与重症肌无力相关的严重疾病发生率较高。
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