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Mapping lung function in late-onset Pompe disease using label-free functional MRI.
Lina Tan1,2,3, Alexandra L Wagner1,2,4, Rafael Heiss5
1Department of Medicine 3 - Rheumatology and Immunology, University Hospital Erlangen, Friedrich-Alexander-Universität (FAU) Erlangen-Nürnberg, Erlangen 91054, Germany.
Phase-resolved functional lung (PREFUL) MRI is a feasible, non-invasive tool for assessing lung function in late-onset Pompe disease (LOPD). It detects ventilation impairments, offering a needed alternative to spirometry for monitoring respiratory health in LOPD patients.
Area of Science:
- Medical Imaging
- Pulmonology
- Neuromuscular Disorders
Background:
- Pompe disease (PD) is a metabolic myopathy causing progressive muscle weakness.
- Late-onset Pompe disease (LOPD) patients often die from respiratory failure due to respiratory muscle weakness.
- Current monitoring via spirometry is effort-dependent and not suitable for all LOPD patients.
Purpose of the Study:
- To evaluate the feasibility and efficacy of Phase-resolved functional lung (PREFUL) MRI in LOPD patients.
- To identify ventilation-related functional impairments in LOPD.
- To explore differences in lung function based on non-invasive ventilation (NIV) status.
Main Methods:
- Prospective pilot study involving ten LOPD patients and ten healthy controls.
- Utilized 0.55 T PREFUL MRI for non-invasive, label-free lung function assessment during free breathing.
- Compared PREFUL MRI findings between LOPD patients and controls, and analyzed effects of NIV status.
Main Results:
- PREFUL MRI was feasible in LOPD patients and healthy controls.
- The study detected significant ventilation-related functional impairments in LOPD patients compared to controls.
- Lung function differences were observed correlating with non-invasive ventilation (NIV) status in LOPD patients.
Conclusions:
- PREFUL MRI is a viable, non-invasive imaging biomarker for assessing respiratory dysfunction in LOPD.
- This method provides effort-independent lung function evaluation, addressing limitations of spirometry.
- PREFUL MRI may help monitor disease progression and treatment efficacy in LOPD and other neuromuscular diseases.
