A new CF-specific MRI-Score: can it predict loss of lung function?
Ilias Tsiflikas1, Matthias Teufel1, Sabrina Fleischer1, Dominik Hartl2, Konstantin Nikolaou1, and Juergen F Schaefer1

1Diagnostic and Interventional Radiology, University Hospital of Tuebingen, Tübingen, Germany, 2Pediatrics I - CF Center, University Hospital of Tuebingen, Tübingen, Germany

Synopsis

The study successfully evaluated a new developed CF-specific MRI score. Our results show that the MRI-Score can predict the loss of pulmonary function. Thus, our findings may help that MRI can serve as a novel predictive marker for loss of lung function in CF and thereby help to tailor individualized monitoring and treatment strategies.

Introduction

Lung imaging is an essential part of the diagnostic work-up in CF patients with Magnetic Resonance Imaging (MRI) representing a novel approach to characterize lung disease. MRI enables beside morphological assessment also a functional evaluation and is preferred in young patients because of absence of radiation. Nevertheless the predictive value of MRI in cystic fibrosis (CF) has not been established.

Purpose

To evaluate usefulness of a novel MRI-Score as a prognostic biomarker for the clinical course of CF.

Methods

Sixty-one CF-patients (mean age 12.9 ± 4.7 y) were examined using an optimized, advanced MRI-protocol for lung imaging including morphological sequences and very short TE PDw Flash 2D for functional evaluation without the use of contrast medium (Fig 1). Further all patients had a pulmonary function testing (PFT) performed within 14 days of the MRI-examination, as well as PFT follow-up over a period of at least 2 years. All MRI-examinations were analyzed by three blinded raters by the means of a self-developed MRI-Score and compared to the Helbich-Bhalla score (HB-S) (Ref 1). To evaluate loss of FEV1, a linear regression analysis over a period of two years was performed.

Results

HB-S and MRI-Score showed a moderate inverse correlation with FEV1 (-0.58 and -0.62, respectively). The best correlation for single categories of HB-S was severity and extent of bronchiectasis (-0.56) and for MRI-Score centrilobular opacity and air trapping (-0.69 and -0.62). Our optimized MRI-Score was able to predict loss of FEV1 within 2 years. Particularly, after excluding patients with high normal (> 99%) or very low values (<45%) of FEV1 at baseline the extent of centrilobular opacity was highly significant associated with a more severe loss of FEV1 in children with CF.

Conclusion

Our new CF-specific MRI-Score predicts loss of pulmonary function in children with CF. Further prospective evaluations of the new score are necessary to confirm its clinical usefulness.

Acknowledgements

No acknowledgement found.

References

1. Helbich TH, Heinz-Peer G, Eichler I, Wunderbaldinger P, Gotz M, Wojnarowski C, et al. Cystic fibrosis: CT assessment of lung involvement in children and adults. Radiology. 1999 Nov;213(2):537-44. PubMed PMID: 10551238. Epub 1999/11/07. eng.

Figures

Fig 1: Institutional pulmonary MRI protocol for CF patients including a) transverse PDw Flash 3D with breath-hold (9-12s), b) coronal fat-saturated T2w TSE with ECG-Triggering and respiratory navigation, and c) + d) sagittal very short TE (0.8ms) PDw Flash 2D in submaximal in- and expiration of the c) right lung and d) left lung.



Proc. Intl. Soc. Mag. Reson. Med. 24 (2016)
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