Department of Neuroscience, ALS Center, "Rita Levi Montalcini", University of Turin, Via Cherasco 15, 1026, Turin, Italy.
SC Pneumologia, AOU Città della Salute e della Scienza of Turin, Turin, Italy.
J Neurol. 2020 Jun;267(6):1615-1621. doi: 10.1007/s00415-020-09751-1. Epub 2020 Feb 12.
To compare the prognostic role of FVC and SVC at diagnosis in amyotrophic lateral sclerosis (ALS) patients.
We included all patients from the Piemonte and Valle D'Aosta ALS register (PARALS) who had been diagnosed with ALS between 1995 and 2015 and underwent spirometry at diagnosis. Survival was considered as time to death/tracheostomy; to assess the prognostic value in typical trial timeframes, survival at 12 and 18 months was calculated too. Cox proportional hazard regression models adjusted by sex, age at diagnosis, diagnostic delay, onset site, and ALSFRS-R total score at the moment of diagnosis were used to assess the prognostic role of FVC and SVC.
A total of 795 ALS patients underwent spirometry at diagnosis during the study period. Four hundred and sixteen (52.3%) performed both FVC and SVC, whereas the others performed FVC only. FVC and SVC values were highly correlated (r = 0.92, p < 0.001) in the overall population and slightly less correlated in patients with bulbar onset (r = 0.86, p < 0.001). Both FVC and SVC proved to have a prognostic role with comparable hazard ratios (HRs) (HR 1.83, 95% CI 1.48-2.27 and 1.88, 95% CI 1.51-2.33, respectively). When considering typical trial timeframes, HRs remained similar and were inversely proportional to FVC and SVC values.
FVC and SVC at diagnosis can be used interchangeably as independent predictors of survival in both clinical and research settings.
比较诊断时 FVC 和 SVC 在肌萎缩侧索硬化症（ALS）患者中的预后作用。
我们纳入了所有 1995 年至 2015 年间在皮埃蒙特和瓦莱达奥斯塔 ALS 登记处（PARALS）诊断为 ALS 并在诊断时接受肺活量测定的患者。将生存定义为死亡/气管切开的时间；为了评估在典型试验时间框架中的预后价值，还计算了 12 个月和 18 个月的生存率。使用 Cox 比例风险回归模型，通过性别、诊断时的年龄、诊断延迟、发病部位和诊断时的 ALSFRS-R 总分进行调整，以评估 FVC 和 SVC 的预后作用。
在研究期间，共有 795 名 ALS 患者在诊断时接受了肺活量测定。其中 416 名（52.3%）同时进行了 FVC 和 SVC 测定，而其他患者仅进行了 FVC 测定。FVC 和 SVC 值在整个人群中高度相关（r=0.92，p<0.001），在延髓发病的患者中相关性稍低（r=0.86，p<0.001）。FVC 和 SVC 均具有预后作用，风险比（HR）相似（HR 1.83，95% CI 1.48-2.27 和 1.88，95% CI 1.51-2.33）。在考虑典型试验时间框架时，HR 仍然相似，并且与 FVC 和 SVC 值成反比。
在临床和研究环境中，诊断时的 FVC 和 SVC 可以互换使用，作为生存的独立预测因子。
