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Prognostic role of slow vital capacity in amyotrophic lateral sclerosis

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Abstract

Objectives

To compare the prognostic role of FVC and SVC at diagnosis in amyotrophic lateral sclerosis (ALS) patients.

Methods

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.

Results

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.

Discussion

FVC and SVC at diagnosis can be used interchangeably as independent predictors of survival in both clinical and research settings.

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Acknowledgements

The project has been supported by Cytokinetics Inc. research Grant; AntC thanks Magnetto Foundation. This study was supported by Ministero dell’Istruzione, dell’Università e della Ricerca–MIUR project “Dipartimenti di Eccellenza 2018–2022” to Department of Neuroscience “Rita Levi Montalcini”.

Author information

Author notes

  1. Andrea Calvo and Rosario Vasta equally contributed to the paper.

  2. Fabrizio D’Ovidio and Umberto Manera equally contributed to the paper.

Authors and Affiliations

  1. Department of Neuroscience, ALS Center, “Rita Levi Montalcini”, University of Turin, Via Cherasco 15, 1026, Turin, Italy

    Andrea Calvo, Rosario Vasta, Cristina Moglia, Enrico Matteoni, Antonio Canosa, Claudio La Mancusa, Adriano Chiò, Fabrizio D’Ovidio & Umberto Manera

  2. SC Pneumologia, AOU Città della Salute e della Scienza of Turin, Turin, Italy

    Alessio Mattei

  3. Presidio ASL4, Pneumologia, Turin, Lanzo Torinese, Italy

    Luana Focaraccio

  4. ALS Center, Ospedale Maggiore della Carità, Novara, Italy

    Letizia Mazzini

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  1. Andrea Calvo
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Contributions

AC, AC and CM contributed to fund raising, study conception and design, interpretation of results and revision of the manuscript. AM, LF, LM, AC, EM, and CLM contributed to data collection and revision of the manuscript. RV, UM and FD contributed to study design, data analysis, interpretation of results, writing and revision of the manuscript.

Corresponding author

Correspondence to Andrea Calvo.

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Conflicts of interest

ACh reports personal fees from Biogen Idec, Cytokinetics, Mitsubishi Tanabe, and Neuraltus, outside the submitted work; other authors have no conflicts of interest.

Ethical approval

The study was approved by the ethical committee of the centers involved. Databases were treated according to the Italian privacy regulations. Patients signed a written informed consent. Anonymized data will be shared on request from any qualified investigator.

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Calvo, A., Vasta, R., Moglia, C. et al. Prognostic role of slow vital capacity in amyotrophic lateral sclerosis. J Neurol 267, 1615–1621 (2020). https://doi.org/10.1007/s00415-020-09751-1

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  • DOI: https://doi.org/10.1007/s00415-020-09751-1

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