New Severity Assessment in Cystic Fibrosis: Signal Intensity and Lung Volume Compared to LCI and FEV: Preliminary Results

Overview

Journal Eur Radiol

Specialty Radiology

Date 2019 Nov 16

PMID 31728685

Citations 2

Authors

Sabrina Fleischer

Mareen Sarah Kraus

Sergios Gatidis

Winfried Baden

Andreas Hector

Dominik Hartl

Ilias Tsiflikas

Juergen Frank Schaefer

Affiliations

Soon will be listed here.

Abstract

Objectives: Magnetic resonance imaging (MRI) aids diagnosis in cystic fibrosis (CF) but its use in quantitative severity assessment is under research. This study aims to assess changes in signal intensity (SI) and lung volumes (Vol) during functional MRI and their use as a severity assessment tool in CF patients.

Methods: The CF intra-hospital standard chest 1.5 T MRI protocol comprises of very short echo-time sequences in submaximal in- and expiration for functional information. Quantitative measurements (Vol/SI at in- and expiration, relative differences (Vol_delta/SI_delta), and cumulative histograms for normalized SI values across the expiratory lung volume) were assessed for correlation to pulmonary function: lung clearance index (LCI) and forced expiratory volume in 1 s (FEV).

Results: In 49 patients (26 male, mean age 17 ± 7 years) significant correlation of Vol_delta and SI_delta (R = 0.86; p < 0.0001) during respiration was observed. Individual cumulated histograms enabled severity disease differentiation (mild, severe) to be visualized (defined by functional parameter: LCI > 10). The expiratory volume at a relative SI of 100% correlated significantly to LCI (R = 0.676 and 0.627; p < 0.0001) and FEV (R = - 0.847 and - 0.807; p < 0.0001). Clustering patients according to Vol_SI_100 showed that an amount of ≤ 4% was related to normal, while an amount of > 4% was associated with pathological pulmonary function values.

Conclusion: Functional pulmonary MRI provides a radiation-free severity assessment tool and can contribute to early detection of lung impairment in CF. Lung volume with SI below 100% of the inspiratory volume represents overinflated tissue; an amount of 4% of the expiratory lung volume was a relevant turning point.

Key Points: • Signal intensity and lung volumes are used as potential metric parameters for lung impairment. • Quantification of trapped air impacts on therapy management. • Functional pulmonary MRI can contribute to early detection of lung impairment.

Citing Articles

Implementation and evaluation of ultra-low dose CT in early cystic fibrosis lung disease.

Bayfield K, Weinheimer O, Boyton C, Fitzpatrick R, Middleton A, Kennedy B Eur Respir J. 2023; 62(1).

PMID: 37385656 PMC: 10327540. DOI: 10.1183/13993003.00286-2023.

State-of-the-art review of lung imaging in cystic fibrosis with recommendations for pulmonologists and radiologists from the "iMAging managEment of cySTic fibROsis" (MAESTRO) consortium.

Ciet P, Bertolo S, Ros M, Casciaro R, Cipolli M, Colagrande S Eur Respir Rev. 2022; 31(163).

PMID: 35321929 PMC: 9489084. DOI: 10.1183/16000617.0173-2021.

References

Bauman G, Puderbach M, Heimann T, Kopp-Schneider A, Fritzsching E, Mall M . Validation of Fourier decomposition MRI with dynamic contrast-enhanced MRI using visual and automated scoring of pulmonary perfusion in young cystic fibrosis patients. Eur J Radiol. 2013; 82(12):2371-7. DOI: 10.1016/j.ejrad.2013.08.018. View

Kraemer R, Blum A, Schibler A, Ammann R, Gallati S . Ventilation inhomogeneities in relation to standard lung function in patients with cystic fibrosis. Am J Respir Crit Care Med. 2004; 171(4):371-8. DOI: 10.1164/rccm.200407-948OC. View

Boss A, Schaefer S, Martirosian P, Claussen C, Schick F, Schaefer J . Magnetic resonance imaging of lung tissue: influence of body positioning, breathing and oxygen inhalation on signal decay using multi-echo gradient-echo sequences. Invest Radiol. 2008; 43(6):433-8. DOI: 10.1097/RLI.0b013e3181690191. View

Altes T, Meyer C, Mata J, Froh D, Paget-Brown A, Teague W . Hyperpolarized helium-3 magnetic resonance lung imaging of non-sedated infants and young children: a proof-of-concept study. Clin Imaging. 2017; 45:105-110. DOI: 10.1016/j.clinimag.2017.04.004. View

Horsley A . Lung clearance index in the assessment of airways disease. Respir Med. 2009; 103(6):793-9. DOI: 10.1016/j.rmed.2009.01.025. View

Tepper L, Ciet P, Caudri D, Quittner A, Utens E, Tiddens H . Validating chest MRI to detect and monitor cystic fibrosis lung disease in a pediatric cohort. Pediatr Pulmonol. 2015; 51(1):34-41. DOI: 10.1002/ppul.23328. View

Kligerman S, Henry T, Lin C, Franks T, Galvin J . Mosaic Attenuation: Etiology, Methods of Differentiation, and Pitfalls. Radiographics. 2015; 35(5):1360-80. DOI: 10.1148/rg.2015140308. View

Kanhere N, Couch M, Kowalik K, Zanette B, Rayment J, Manson D . Correlation of Lung Clearance Index with Hyperpolarized Xe Magnetic Resonance Imaging in Pediatric Subjects with Cystic Fibrosis. Am J Respir Crit Care Med. 2017; 196(8):1073-1075. DOI: 10.1164/rccm.201611-2228LE. View

Bauman G, Pusterla O, Santini F, Bieri O . Dynamic and steady-state oxygen-dependent lung relaxometry using inversion recovery ultra-fast steady-state free precession imaging at 1.5 T. Magn Reson Med. 2017; 79(2):839-845. DOI: 10.1002/mrm.26739. View

10.

Dorlochter L, Nes H, Fluge G, Rosendahl K . High resolution CT in cystic fibrosis--the contribution of expiratory scans. Eur J Radiol. 2003; 47(3):193-8. DOI: 10.1016/s0720-048x(02)00097-9. View

11.

OSullivan B, Freedman S . Cystic fibrosis. Lancet. 2009; 373(9678):1891-904. DOI: 10.1016/S0140-6736(09)60327-5. View

12.

Biederer J, Heussel C, Puderbach M, Wielpuetz M . Functional magnetic resonance imaging of the lung. Semin Respir Crit Care Med. 2014; 35(1):74-82. DOI: 10.1055/s-0033-1363453. View

13.

Fuchs S, Ellemunter H, Eder J, Mellies U, Grosse-Onnebrink J, Tummler B . Feasibility and variability of measuring the Lung Clearance Index in a multi-center setting. Pediatr Pulmonol. 2011; 47(7):649-57. DOI: 10.1002/ppul.21610. View

14.

Leutz-Schmidt P, Stahl M, Sommerburg O, Eichinger M, Puderbach M, Schenk J . Non-contrast enhanced magnetic resonance imaging detects mosaic signal intensity in early cystic fibrosis lung disease. Eur J Radiol. 2018; 101:178-183. DOI: 10.1016/j.ejrad.2018.02.023. View

15.

Stahl M, Wielputz M, Graeber S, Joachim C, Sommerburg O, Kauczor H . Comparison of Lung Clearance Index and Magnetic Resonance Imaging for Assessment of Lung Disease in Children with Cystic Fibrosis. Am J Respir Crit Care Med. 2016; 195(3):349-359. DOI: 10.1164/rccm.201604-0893OC. View

16.

Plathow C, Schoebinger M, Fink C, Ley S, Puderbach M, Eichinger M . Evaluation of lung volumetry using dynamic three-dimensional magnetic resonance imaging. Invest Radiol. 2005; 40(3):173-9. DOI: 10.1097/00004424-200503000-00007. View

17.

Fuchs S, Eder J, Ellemunter H, Gappa M . Lung clearance index: normal values, repeatability, and reproducibility in healthy children and adolescents. Pediatr Pulmonol. 2009; 44(12):1180-5. DOI: 10.1002/ppul.21093. View

18.

Bankier A, ODonnell C, Mai V, Storey P, De Maertelaer V, Edelman R . Impact of lung volume on MR signal intensity changes of the lung parenchyma. J Magn Reson Imaging. 2004; 20(6):961-6. DOI: 10.1002/jmri.20198. View

19.

Sheikh K, Guo F, Capaldi D, Ouriadov A, Eddy R, Svenningsen S . Ultrashort echo time MRI biomarkers of asthma. J Magn Reson Imaging. 2016; 45(4):1204-1215. DOI: 10.1002/jmri.25503. View

20.

Gustafsson P, Aurora P, Lindblad A . Evaluation of ventilation maldistribution as an early indicator of lung disease in children with cystic fibrosis. Eur Respir J. 2003; 22(6):972-9. DOI: 10.1183/09031936.03.00049502. View