Spirometry correlates with physical activity in patients with Duchenne muscular dystrophy

BackgroundDuchenne muscular dystrophy (DMD) is associated with progressive muscle weakness and respiratory decline. To date, studies have focused on respiratory decline and loss of ambulation as a metric of loss of skeletal muscle strength. However, new functional measures can assess skeletal muscle disease regardless of ambulatory status. The relationship between these tests and concurrent lung function is currently unexplored. ObjectiveTo assess the correlation between spirometry measurements and functional muscle assessments such as accelerometry and quantitative muscle testing (QMT). MethodsEnrolled patients with DMD underwent accelerometry and QMT at study clinic visits. Any pulmonary function testing within 6 months of visit was obtained from the electronic medical record. The Spearman correlation coefficient was used to assess the relationship between spirometry and functional muscle testing. ResultsForced vital capacity (FVC) and forced expiratory volume in 1 s (FEV1) demonstrated the strongest correlation with accelerometry. Both FVC and FEV1 showed a similar relationship to accelerometry when activity was divided into intensity categories, with low intensity and moderate-to-vigorous activity categories showing the strongest correlation. Maximal expiratory pressure (MEP) and FVC showed the most robust correlations with total QMT (p < 0.001 and p < 0.01, respectively). ConclusionLung function, specifically FVC percent predicted and FEV1%p, shows a good correlation with upper and lower extremity skeletal muscle functional testing such as accelerometry and QMT.

Automated summary

Current studies have mainly focused on respiratory decline and loss of ambulation as indicators of skeletal muscle strength loss in Duchenne muscular dystrophy (DMD). However, this study found that measures of lung function, specifically forced vital capacity (FVC) and forced expiratory volume in 1 s (FEV1), showed a strong correlation with functional muscle assessments such as accelerometry and quantitative muscle testing (QMT). Maximal expiratory pressure (MEP) and FVC demonstrated the most robust correlations with total QMT.