• Megan E Angliss, Kiara D Sclip, and Leanne Gauld.
• Respiratory Medicine, Children's Health Queensland Hospital and Health Service, South Birsbane, Queensland, Australia.
• BMJ Open Respir Res. 2020 Feb 1; 7 (1).

BackgroundUse of non-invasive ventilation (NIV) in adolescents with Duchenne muscular dystrophy (DMD) has increased with concomitant extended survival.AimTo describe lung function (LF) changes with NIV in adolescents with DMD and to assess differences between Steroid Users and Steroid Naïve subjects.MethodA retrospective cohort of adolescents with DMD initiating NIV over 10 years was conducted. Serial LF before and after NIV initiation was collated. Use of systemic glucocorticosteroids, adherence to NIV and presence of cardiac disease were assessed.ResultsTwenty-nine men started NIV, median age 14.66 years (IQR 2.35, 10.47-17.96). Nine were Steroid Users and eight were Steroid Naïve. Indications for NIV were apnoea-hypopnoea index >5 and/or nocturnal hypoventilation. LF is better (forced vital capacity (FVC) z-score -3.26 vs -5.41, p < 0.02) and decline slower (FVC z-score -0.58 per annum (pa) vs -0.68 pa, p<0.001) in Steroid Users compared with Steroid Naïve subjects. Following NIV initiation, FVC z-score decline slowed for the whole (-0.72 pa (95% CI -0.79 to 0.64) to -0.46 pa (95% CI -0.54 to 0.38) p < 0.001) and Steroid Naïve groups (-0.74 (95% CI -0.85 to 0.63) to -0.44 pa (95% CI -0.56 to 0.32) p < 0.001) but accelerated in the Steroid User group (-0.56 (95% CI -0.70 to 0.42) to -0.75 pa (95% CI -0.89 to 0.61) p < 0.001). Adherence to NIV and cardiac disease did not impact decline.ConclusionOverall, LF decline is reduced on NIV. Steroid Naïve patients have lower LF and faster decline, which slows following NIV initiation. An accelerated LF decline was seen on NIV in Steroid Users which requires further prospective research.© Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.

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