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Characterizing Expiratory Respiratory Muscle Degeneration in Duchenne Muscular Dystrophy Using MRI. | LitMetric

AI Article Synopsis

  • Expiratory muscle weakness and impaired airway clearance are early indicators of respiratory issues in Duchenne muscular dystrophy (DMD), a condition where muscle cells deteriorate and are replaced by fat tissue.
  • A study was conducted to track changes in fatty infiltration in abdominal expiratory muscles over time using MRI, correlating it with clinical measures of expiratory function like peak cough flow and lung pressures.
  • Findings revealed that specific muscles, particularly the internal oblique, show faster degeneration and accumulation of fat, significantly affecting respiratory capabilities, especially in untreated individuals compared to those receiving corticosteroid treatment.

Article Abstract

Background: Expiratory muscle weakness and impaired airway clearance are early signs of respiratory dysfunction in Duchenne muscular dystrophy (DMD), a degenerative muscle disorder in which muscle cells are damaged and replaced by fibrofatty tissue. Little is known about expiratory muscle pathology and its relationship to cough and airway clearance capacity; however, the level of muscle replacement by fat can be estimated using MRI and expressed as a fat fraction (FF).

Research Question: How does abdominal expiratory muscle fatty infiltration change over time in DMD and relate to clinical expiratory function?

Study Design And Methods: Individuals with DMD underwent longitudinal MRI of the abdomen to determine FF in the internal oblique, external oblique, and rectus abdominis expiratory muscles. FF data were used to estimate a model of expiratory muscle degeneration by using nonlinear mixed effects and a cumulative distribution function. FVC, maximal inspiratory and expiratory pressures, and peak cough flow were collected as clinical correlates to MRI.

Results: Forty individuals with DMD (aged 6-18 years at baseline) participated in up to five visits over 36 months. Modeling estimated the internal oblique progresses most quickly and reached 50% replacement by fat at a mean patient age of 13.0 years (external oblique, 14.0 years; rectus abdominis, 16.2 years). Corticosteroid-untreated individuals (n = 4) reached 50% muscle replacement by fat 3 to 4 years prior to treated individuals. Individuals with mild clinical dystrophic phenotypes (n = 3) reached 50% muscle replacement by fat 4 to 5 years later than corticosteroid-treated individuals. Internal and external oblique FFs near 50% were associated with maximal expiratory pressures < 60 cm HO and peak cough flows < 270 L/min.

Interpretation: These data improve understanding of the early phase of respiratory compromise in DMD, which typically presents as airway clearance dysfunction prior to the onset of hypoventilation, and links expiratory muscle fatty infiltration to pulmonary function measures.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9160975PMC
http://dx.doi.org/10.1016/j.chest.2021.08.078DOI Listing

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