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Letter to the Editor| Volume 20, ISSUE 3, P552-554, May 2021

Dornase alfa and rate of lung function decline in European patients with cystic fibrosis: A retrospective registry cohort study

Published:August 21, 2020DOI:https://doi.org/10.1016/j.jcf.2020.08.004
Dornase alfa and rate of lung function decline in European patients with cystic fibrosis: A retrospective registry cohort study
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      Dornase alfa, a treatment that hydrolyses DNA, is approved as a therapy for patients with Cystic Fibrosis (CF). This mucolytic effect was shown in a pivotal clinical trial to improve lung function and reduce the frequency of CF pulmonary exacerbations [
      • Fuchs H.J.
      • Borowitz D.S.
      • Christiansen D.H.
      • Morris E.M.
      • Nash M.L.
      • Ramsey B.W.
      • et al.
      Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis. The Pulmozyme Study Group.
      N Engl J Med. 1994; 331: 637-642
      ]. Subsequent longitudinal studies of the impact of dornase alfa on lung function in United States’ and United Kingdom cohorts have shown additional long-term benefits including reductions in lung function decline, although these findings vary owing to differences in study design [
      • Hodson M.E.
      • McKenzie S.
      • Harms H.K.
      • Koch C.
      • Mastella G.
      • Navarro J.
      • et al.
      Dornase alfa in the treatment of cystic fibrosis in Europe: a report from the epidemiologic registry of cystic fibrosis.
      Pediatr Pulmonol. 2003; 36: 427-432
      ,
      • Konstan M.W.
      • Wagener J.S.
      • Pasta D.J.
      • Millar S.J.
      • Jacobs J.R.
      • Yegin A.
      • et al.
      Clinical use of dornase alpha is associated with a slower rate of FEV1 decline in cystic fibrosis.
      Pediatr Pulmonol. 2011; 46: 545-553
      ,
      • Newsome S.J.
      • Daniel R.M.
      • Carr S.B.
      • Bilton D.
      • Keogh R.H.
      Investigating the effects of long-term dornase alfa use on lung function using registry data.
      J Cyst Fibros. 2019; 18: 110-117
      ]. In this study, we examined whether lung function decline is impacted by chronic dornase alfa treatment in subjects across Europe enrolled in the European Cystic Fibrosis Society Patient Registry (ECFSPR).
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      References

        • Fuchs H.J.
        • Borowitz D.S.
        • Christiansen D.H.
        • Morris E.M.
        • Nash M.L.
        • Ramsey B.W.
        • et al.
        Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis. The Pulmozyme Study Group.
        N Engl J Med. 1994; 331: 637-642
        View in Article
        • Hodson M.E.
        • McKenzie S.
        • Harms H.K.
        • Koch C.
        • Mastella G.
        • Navarro J.
        • et al.
        Dornase alfa in the treatment of cystic fibrosis in Europe: a report from the epidemiologic registry of cystic fibrosis.
        Pediatr Pulmonol. 2003; 36: 427-432
        View in Article
        • Konstan M.W.
        • Wagener J.S.
        • Pasta D.J.
        • Millar S.J.
        • Jacobs J.R.
        • Yegin A.
        • et al.
        Clinical use of dornase alpha is associated with a slower rate of FEV1 decline in cystic fibrosis.
        Pediatr Pulmonol. 2011; 46: 545-553
        View in Article
        • Newsome S.J.
        • Daniel R.M.
        • Carr S.B.
        • Bilton D.
        • Keogh R.H.
        Investigating the effects of long-term dornase alfa use on lung function using registry data.
        J Cyst Fibros. 2019; 18: 110-117
        View in Article

      Article info

      Publication history

      Published online: August 21, 2020
      Accepted: August 10, 2020
      Received: August 7, 2020

      Identification

      DOI: https://doi.org/10.1016/j.jcf.2020.08.004

      Copyright

      © 2020 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

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