Early NIV is associated with accelerated lung function decline in Duchenne muscular dystrophy treated with glucocorticosteroids.

BACKGROUND

Use of non-invasive ventilation (NIV) in adolescents with Duchenne muscular dystrophy (DMD) has increased with concomitant extended survival.

AIM

To describe lung function (LF) changes with NIV in adolescents with DMD and to assess differences between and subjects.

METHOD

A 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.

RESULTS

Twenty-nine men started NIV, median age 14.66 years (IQR 2.35, 10.47-17.96). Nine were and eight were . 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 compared with 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 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 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.

CONCLUSION

Overall, LF decline is reduced on NIV. patients have lower LF and faster decline, which slows following NIV initiation. An accelerated LF decline was seen on NIV in which requires further prospective research.