Myasthenic crisis demanding mechanical ventilation: A multicenter analysis of 250 cases
Myasthenic crisis demanding mechanical ventilation: A multicenter analysis of 250 cases
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DOI：
10.1212/wnl.0000000000008688
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发表时间：
2020-01-21
期刊：
影响因子：
9.9
通讯作者：
Stetefeld, Henning R.
中科院分区：
文献类型：
--
作者：
Neumann, Bernhard;Angstwurm, Klemens;Stetefeld, Henning R.
目的探讨重症肌无力危象(MC)患者的人口学特征、临床特征、治疗方案和转归。方法对2006-2015年间在德国进行的一项多中心回顾性研究中出现的MC患者进行分析。结果12个参与中心共250例。危机发生时的中位年龄为72岁。中位MV持续时间为12天。呼吸机持续时间(>；15天)取决于年龄(p=0.0001)、迟发性重症肌无力(MG)、危象前美国重症肌无力基金会分级(p=0.0001，优势比[OR]=无穷大)、合并症数量(>；3合并：p=0.002，OR2.99)、肺炎(p=0.0001，OR3.13)和复苏(p=0.0008，OR9.15)。20.5%的幸存者在出院时需要MV。早发性重症肌无力(P=0.0001，OR0.21)、胸腺增生(P=0.002，OR 0)、无创机械通气试验成功的患者更有可能在15天内接受呼吸机治疗。无创通气率38%，机械通气量明显减少(P=0.001)，重症监护室住院天数明显减少(P=0.01)。当机械通气持续时间和在ICU的停留时间延长时，静脉免疫球蛋白、血浆置换和免疫吸附更有可能按顺序合并(p=0.0503，OR2.05)。接受血浆置换或免疫吸附作为一线治疗的患者需要有创呼吸机的次数显著减少(p=0.003)。住院死亡率为12%，这与合并症(>；3)的数量以及急性呼吸窘迫综合征和复苏等并发症显著相关。死亡的主要原因是多器官衰竭，主要是由于败血症。结论尽管我们的研究中年龄较高，疾病负担较高，但MC的死亡率和病程与以前的报道相当。预防和治疗并发症以及专门的神经重症监护是改善结果的基石。
ObjectiveTo determine demographic characteristics, clinical features, treatment regimens, and outcome of myasthenic crisis (MC) requiring mechanical ventilation (MV).MethodsAnalysis of patients who presented with MC between 2006 and 2015 in a German multicenter retrospective study.ResultsWe identified 250 cases in 12 participating centers. Median age at crisis was 72 years. Median duration of MV was 12 days. Prolonged ventilation (>15 days) depended on age (p = 0.0001), late-onset myasthenia gravis (MG), a high Myasthenia Gravis Foundation of America Class before crisis (p = 0.0001 for IVb, odds ratio [OR] = infinite), number of comorbidities (>3 comorbidities: p = 0.002, OR 2.99), pneumonia (p = 0.0001, OR 3.13), and resuscitation (p = 0.0008, OR 9.15). MV at discharge from hospital was necessary in 20.5% of survivors. Patients with early-onset MG (p = 0.0001, OR 0.21), thymus hyperplasia (p = 0.002, OR 0), and successful noninvasive ventilation trial were more likely to be ventilated for less than 15 days. Noninvasive ventilation in 92 cases was sufficient in 38%, which was accompanied by a significantly shorter duration of ventilation (p = 0.001) and intensive care unit (ICU) stay (p = 0.01). IV immunoglobulins, plasma exchange, and immunoadsorption were more likely to be combined sequentially if the duration of MV and the stay in an ICU extended (p = 0.0503, OR 2.05). Patients who received plasma exchange or immunoadsorption as first-line therapy needed invasive ventilation significantly less often (p = 0.003). In-hospital mortality was 12%, which was significantly associated with the number of comorbidities (>3) and complications such as acute respiratory distress syndrome and resuscitation. Main cause of death was multiorgan failure, mostly due to sepsis.ConclusionMortality and duration of MC remained comparable to previous reports despite higher age and a high disease burden in our study. Prevention and treatment of complications and specialized neurointensive care are the cornerstones in order to improve outcome.