A 3-year prognostic score for adults with cystic fibrosis

Lionelle Nkam (1) , J. Lambert (2) , Aurélien Latouche (3) , G. Bellis (4) , P.-R. Burgel (5, 6, 7, 8) , Mounia Hocine (1)

1 MESuRS - Laboratoire Modélisation, épidémiologie et surveillance des risques sanitaires
2 Hôpital Saint-Louis
3 CEDRIC - Centre d'études et de recherche en informatique et communications
4 INED - Institut national d'études démographiques
5 Service de pneumologie [CHU Cochin]
6 AP-HP - Assistance publique - Hôpitaux de Paris (AP-HP)
7 UPD5 - Université Paris Descartes - Paris 5
8 USPC - Université Sorbonne Paris Cité

Background: Therapeutic progress in patients with cystic fibrosis (CF) has resulted in improved prognosis over the past decades. We aim to reevaluate prognostic factors of CF and provide a prognostic score to predict the risk of death or lung transplantation (LT) within a 3-year period in adult patients. Methods: We developed a logistic model using data from the French CF Registry and combined the coefficients into a prognostic score. The discriminative abilities of the model and the prognostic score were assessed by c-statistic. The prognostic score was validated using a 10-fold cross validation. Results: The risk of death or LT within 3 years was related to eight characteristics. The development and the validation provided excellent results for the prognostic score; the c-statistic was 0.91 and 0.90 respectively. Conclusion: The score developed to predict 3-year death or LT in adults with CF might be useful for clinicians to identify patients requiring specialized evaluation for LT.

Keywords: Prognostic score; Logistic model; Cystic fibrosis; Prognostic factors; Registry data

Domains: Pulmonology and respiratory tract; Santé publique et épidémiologie

Lionelle Nkam, J. Lambert, Aurélien Latouche, G. Bellis, P.-R. Burgel, et al.. A 3-year prognostic score for adults with cystic fibrosis. Journal of Cystic Fibrosis, 2017, 16 (6), pp.702-708. ⟨10.1016/j.jcf.2017.03.004⟩. ⟨hal-02080563⟩