Pinto Susana, de Carvalho Mamede
a Instituto de Medicina Molecular and Institute of Physiology, Faculty of Medicine , University of Lisbon , Lisbon , Portugal.
b Department of Pharmacology and Clinical Neurosciences, Division of Clin Neurophysiology , Umeå University, Norrlands universitetssjukhus , Umeå Sweden , and.
Amyotroph Lateral Scler Frontotemporal Degener. 2017 Nov;18(7-8):528-533. doi: 10.1080/21678421.2017.1354995. Epub 2017 Jul 25.
Slow (SVC) and forced (FVC) vital capacities are the most used pulmonary function tests in amyotrophic lateral sclerosis (ALS). It is unknown if they equally predict survival in ALS. The aim of the present study was to compare both measures in predicting survival in this disease.
Consecutive definite/probable ALS patients (2000-2014) in whom respiratory tests were performed at baseline and four months later were included. All patients were evaluated with the revised ALS functional rating scale (ALSFRS-R), respiratory (RofALSFRS-R), bulbar (ALSFRSb), upper and lower limb subscores, SVC, FVC, maximal inspiratory (MIP) and expiratory (MEP) pressures. King's functional staging system was applied retrospectively. Survival analysis was carried out by univariate Kaplan-Meier log-rank test. Multivariate Cox proportional hazards model determined significant independent variables.
We included 469 patients (270 males; mean onset age 61.0 ± 11.5 years; mean disease duration from first symptoms to first visit: 15.8 ± 16.1months; 329 spinal and 140 bulbar onset). FVC and SVC were strongly correlated (r = 0.981, p < 0.001). Significant survival prognostic variables (Kaplan-Meier analyses) were onset region, age, disease duration, ALSFRS-R, ALSFRSb, RofALSFRS-R, ALSFRS-R decay, SVC, FVC, MIP, MEP and King's staging (p ≤ 0.01). Final Cox model including the significant variables showed similar results for FVC and SVC (p < 0.001). Moreover, 1% decrease in either predicted values increased death probability by 1.02.
FVC and SVC are inter-changeable in predicting survival in ALS.
慢肺活量（SVC）和用力肺活量（FVC）是肌萎缩侧索硬化症（ALS）中最常用的肺功能测试。目前尚不清楚它们是否同样能预测ALS患者的生存期。本研究旨在比较这两种指标对该疾病生存期的预测能力。
纳入2000年至2014年间连续的确诊/疑似ALS患者，这些患者在基线时和四个月后进行了呼吸测试。所有患者均采用修订的ALS功能评定量表（ALSFRS-R）、呼吸部分（RofALSFRS-R）、延髓部分（ALSFRSb）、上肢和下肢子评分、SVC、FVC、最大吸气压力（MIP）和最大呼气压力（MEP）进行评估。回顾性应用King功能分期系统。通过单因素Kaplan-Meier对数秩检验进行生存分析。多因素Cox比例风险模型确定显著的独立变量。
我们纳入了469例患者（270例男性；平均发病年龄61.0±11.5岁；从首次症状出现到首次就诊的平均病程：15.8±16.1个月；329例脊髓型发病和140例延髓型发病）。FVC和SVC高度相关（r = 0.981，p < 0.001）。显著的生存预后变量（Kaplan-Meier分析）包括发病部位、年龄、病程、ALSFRS-R、ALSFRSb、RofALSFRS-R、ALSFRS-R下降、SVC、FVC、MIP、MEP和King分期（p≤0.01）。包含显著变量的最终Cox模型显示FVC和SVC的结果相似（p < 0.001）。此外，预测值每降低1%，死亡概率增加1.02。
FVC和SVC在预测ALS患者生存期方面具有互换性。
