Skip to main content
Top
Published in: European Radiology 1/2018

01-01-2018 | Magnetic Resonance

A semiquantitative MRI-Score can predict loss of lung function in patients with cystic fibrosis: Preliminary results

Authors: Juergen F. Schaefer, Andreas Hector, Katharina Schmidt, Matthias Teufel, Sabrina Fleischer, Ute Graepler-Mainka, Joachim Riethmueller, Sergios Gatidis, Susanne Schaefer, Konstantin Nikolaou, Dominik Hartl, Ilias Tsiflikas

Published in: European Radiology | Issue 1/2018

Login to get access

Abstract

Objectives

To evaluate the applicability of a semiquantitative MRI scoring system (MR-CF-S) as a prognostic marker for clinical course of cystic fibrosis (CF) lung disease.

Methods

This observational study of a single-centre CF cohort included a group of 61 patients (mean age 12.9 ± 4.7 years) receiving morphological and functional pulmonary MRI, pulmonary function testing (PFT) and follow-up of 2 years. MRI was analysed by three raters using MR-CF-S. The inter-rater agreement, correlation of score categories with forced expiratory volume in 1 s (FEV1) at baseline, and the predictive value of clinical parameters, and score categories was assessed for the whole cohort and a subgroup of 40 patients with moderately impaired lung function.

Results

The inter-rater agreement of MR-CF-S was sufficient (mean intraclass correlation coefficient 0.92). MR-CF-S (−0.62; p < 0.05) and most of the categories significantly correlated with FEV1. Differences between patients with relevant loss of FEV1 (>3%/year) and normal course were only significant for MR-CF-S (p < 0.05) but not for clinical parameters. Centrilobular opacity (CO) was the most promising score category for prediction of a decline of FEV1 (area under curve: whole cohort 0.69; subgroup 0.86).

Conclusions

MR-CF-S is promising to predict a loss of lung function. CO seems to be a particular finding in CF patients with an abnormal course.

Key Points

Lung imaging is essential in the diagnostic work-up of CF patients
MRI serves as a powerful, radiation-free modality in paediatric CF patients
Observational single-centre study showed significant correlation of MR-CF score and FEV 1
MR-CF score is promising in predicting a loss of lung function
Literature
1.
go back to reference Mall MA, Hartl D (2014) CFTR: cystic fibrosis and beyond. European Respiratory Journal 44:1042–54CrossRefPubMed Mall MA, Hartl D (2014) CFTR: cystic fibrosis and beyond. European Respiratory Journal 44:1042–54CrossRefPubMed
2.
go back to reference Cantin AM, Hartl D, Konstan MW, Chmiel JF (2015) Inflammation in cystic fibrosis lung disease: Pathogenesis and therapy. Journal of Cystic Fibrosis 14:419–30CrossRefPubMed Cantin AM, Hartl D, Konstan MW, Chmiel JF (2015) Inflammation in cystic fibrosis lung disease: Pathogenesis and therapy. Journal of Cystic Fibrosis 14:419–30CrossRefPubMed
3.
go back to reference Shah RM, Sexauer W, Ostrum BJ, Fiel SB, Friedman AC (1997) High-resolution CT in the acute exacerbation of cystic fibrosis: evaluation of acute findings, reversibility of those findings, and clinical correlation. AJR American Journal of Roentgenology 169:375–80CrossRefPubMed Shah RM, Sexauer W, Ostrum BJ, Fiel SB, Friedman AC (1997) High-resolution CT in the acute exacerbation of cystic fibrosis: evaluation of acute findings, reversibility of those findings, and clinical correlation. AJR American Journal of Roentgenology 169:375–80CrossRefPubMed
4.
go back to reference de Jong PA, Lindblad A, Rubin L, Hop WC, de Jongste JC, Brink M et al (2006) Progression of lung disease on computed tomography and pulmonary function tests in children and adults with cystic fibrosis. Thorax 61:80–5CrossRefPubMed de Jong PA, Lindblad A, Rubin L, Hop WC, de Jongste JC, Brink M et al (2006) Progression of lung disease on computed tomography and pulmonary function tests in children and adults with cystic fibrosis. Thorax 61:80–5CrossRefPubMed
5.
go back to reference Wielputz MO, Puderbach M, Kopp-Schneider A, Stahl M, Fritzsching E, Sommerburg O et al (2014) Magnetic resonance imaging detects changes in structure and perfusion, and response to therapy in early cystic fibrosis lung disease. American Journal of Respiratory and Critical Care Medicine 189:956–65CrossRefPubMed Wielputz MO, Puderbach M, Kopp-Schneider A, Stahl M, Fritzsching E, Sommerburg O et al (2014) Magnetic resonance imaging detects changes in structure and perfusion, and response to therapy in early cystic fibrosis lung disease. American Journal of Respiratory and Critical Care Medicine 189:956–65CrossRefPubMed
6.
go back to reference Carpio C, Albi G, Rayon-Aledo JC, Alvarez-Sala R, Giron R, Prados C et al (2015) Changes in structural lung disease in cystic fibrosis children over 4 years as evaluated by high-resolution computed tomography. European Radiology. doi:10.1007/s00330-015-3782-4 PubMed Carpio C, Albi G, Rayon-Aledo JC, Alvarez-Sala R, Giron R, Prados C et al (2015) Changes in structural lung disease in cystic fibrosis children over 4 years as evaluated by high-resolution computed tomography. European Radiology. doi:10.​1007/​s00330-015-3782-4 PubMed
7.
go back to reference Tiddens HA, Rosenow T (2014) What did we learn from two decades of chest computed tomography in cystic fibrosis? Pediatric Radiology 44:1490–5CrossRefPubMed Tiddens HA, Rosenow T (2014) What did we learn from two decades of chest computed tomography in cystic fibrosis? Pediatric Radiology 44:1490–5CrossRefPubMed
8.
go back to reference Kuo W, Ciet P, Tiddens HA, Zhang W, Guillerman RP, van Straten M (2014) Monitoring cystic fibrosis lung disease by computed tomography. Radiation risk in perspective. American Journal of Respiratory and Critical Care Medicine 189:1328–36CrossRefPubMed Kuo W, Ciet P, Tiddens HA, Zhang W, Guillerman RP, van Straten M (2014) Monitoring cystic fibrosis lung disease by computed tomography. Radiation risk in perspective. American Journal of Respiratory and Critical Care Medicine 189:1328–36CrossRefPubMed
9.
go back to reference Eichinger M, Optazaite DE, Kopp-Schneider A, Hintze C, Biederer J, Niemann A et al (2012) Morphologic and functional scoring of cystic fibrosis lung disease using MRI. European Journal of Radiology 81:1321–9CrossRefPubMed Eichinger M, Optazaite DE, Kopp-Schneider A, Hintze C, Biederer J, Niemann A et al (2012) Morphologic and functional scoring of cystic fibrosis lung disease using MRI. European Journal of Radiology 81:1321–9CrossRefPubMed
10.
go back to reference Teufel M, Ketelsen D, Fleischer S, Martirosian P, Graebler-Mainka U, Stern M et al (2013) Comparison between high-resolution CT and MRI using a very short echo time in patients with cystic fibrosis with extra focus on mosaic attenuation. Respiration 86:302–11CrossRefPubMed Teufel M, Ketelsen D, Fleischer S, Martirosian P, Graebler-Mainka U, Stern M et al (2013) Comparison between high-resolution CT and MRI using a very short echo time in patients with cystic fibrosis with extra focus on mosaic attenuation. Respiration 86:302–11CrossRefPubMed
11.
12.
go back to reference Veldhoen S, Weng AM, Knapp J, Kunz AS, Stab D, Wirth C et al (2016) Self-gated non-contrast-enhanced functional lung MR imaging for quantitative ventilation assessment in patients with cystic fibrosis. Radiology. doi:10.1148/radiol.2016160355 PubMed Veldhoen S, Weng AM, Knapp J, Kunz AS, Stab D, Wirth C et al (2016) Self-gated non-contrast-enhanced functional lung MR imaging for quantitative ventilation assessment in patients with cystic fibrosis. Radiology. doi:10.​1148/​radiol.​2016160355 PubMed
13.
go back to reference Tepper LA, Ciet P, Caudri D, Quittner AL, Utens EM, Tiddens HA (2016) Validating chest MRI to detect and monitor cystic fibrosis lung disease in a pediatric cohort. Pediatric Pulmonology 51:34–41CrossRefPubMed Tepper LA, Ciet P, Caudri D, Quittner AL, Utens EM, Tiddens HA (2016) Validating chest MRI to detect and monitor cystic fibrosis lung disease in a pediatric cohort. Pediatric Pulmonology 51:34–41CrossRefPubMed
14.
go back to reference Que C, Cullinan P, Geddes D (2006) Improving rate of decline of FEV1 in young adults with cystic fibrosis. Thorax 61:155–7CrossRefPubMed Que C, Cullinan P, Geddes D (2006) Improving rate of decline of FEV1 in young adults with cystic fibrosis. Thorax 61:155–7CrossRefPubMed
15.
go back to reference Konstan MW, Wagener JS, Vandevanter DR, Pasta DJ, Yegin A, Rasouliyan L et al (2012) Risk factors for rate of decline in FEV1 in adults with cystic fibrosis. Journal of Cystic Fibrosis 11:405–11CrossRefPubMedPubMedCentral Konstan MW, Wagener JS, Vandevanter DR, Pasta DJ, Yegin A, Rasouliyan L et al (2012) Risk factors for rate of decline in FEV1 in adults with cystic fibrosis. Journal of Cystic Fibrosis 11:405–11CrossRefPubMedPubMedCentral
16.
go back to reference Konstan MW, Morgan WJ, Butler SM, Pasta DJ, Craib ML, Silva SJ et al (2007) Risk factors for rate of decline in forced expiratory volume in one second in children and adolescents with cystic fibrosis. The Journal of Pediatrics 151:134–9.e1CrossRefPubMed Konstan MW, Morgan WJ, Butler SM, Pasta DJ, Craib ML, Silva SJ et al (2007) Risk factors for rate of decline in forced expiratory volume in one second in children and adolescents with cystic fibrosis. The Journal of Pediatrics 151:134–9.e1CrossRefPubMed
17.
go back to reference Cogen J, Emerson J, Sanders DB, Ren C, Schechter MS, Gibson RL et al (2015) Risk factors for lung function decline in a large cohort of young cystic fibrosis patients. Pediatric Pulmonology 50:763–70CrossRefPubMedPubMedCentral Cogen J, Emerson J, Sanders DB, Ren C, Schechter MS, Gibson RL et al (2015) Risk factors for lung function decline in a large cohort of young cystic fibrosis patients. Pediatric Pulmonology 50:763–70CrossRefPubMedPubMedCentral
19.
go back to reference Terheggen-Lagro S, Truijens N, van Poppel N, Gulmans V, van der Laag J, van der Ent C (2003) Correlation of six different cystic fibrosis chest radiograph scoring systems with clinical parameters. Pediatric Pulmonology 35:441–5CrossRefPubMed Terheggen-Lagro S, Truijens N, van Poppel N, Gulmans V, van der Laag J, van der Ent C (2003) Correlation of six different cystic fibrosis chest radiograph scoring systems with clinical parameters. Pediatric Pulmonology 35:441–5CrossRefPubMed
20.
go back to reference Puderbach M, Eichinger M, Haeselbarth J, Ley S, Kopp-Schneider A, Tuengerthal S et al (2007) Assessment of morphological MRI for pulmonary changes in cystic fibrosis (CF) patients: comparison to thin-section CT and chest x-ray. Investigative Radiology 42:715–25CrossRefPubMed Puderbach M, Eichinger M, Haeselbarth J, Ley S, Kopp-Schneider A, Tuengerthal S et al (2007) Assessment of morphological MRI for pulmonary changes in cystic fibrosis (CF) patients: comparison to thin-section CT and chest x-ray. Investigative Radiology 42:715–25CrossRefPubMed
21.
go back to reference Dournes G, Menut F, Macey J, Fayon M, Chateil JF, Salel M et al (2016) Lung morphology assessment of cystic fibrosis using MRI with ultra-short echo time at submillimeter spatial resolution. European Radiology 26:3811–20CrossRefPubMed Dournes G, Menut F, Macey J, Fayon M, Chateil JF, Salel M et al (2016) Lung morphology assessment of cystic fibrosis using MRI with ultra-short echo time at submillimeter spatial resolution. European Radiology 26:3811–20CrossRefPubMed
22.
go back to reference Sileo C, Corvol H, Boelle PY, Blondiaux E, Clement A, Ducou Le Pointe H (2014) HRCT and MRI of the lung in children with cystic fibrosis: comparison of different scoring systems. Journal of Cystic Fibrosis 13:198–204CrossRefPubMed Sileo C, Corvol H, Boelle PY, Blondiaux E, Clement A, Ducou Le Pointe H (2014) HRCT and MRI of the lung in children with cystic fibrosis: comparison of different scoring systems. Journal of Cystic Fibrosis 13:198–204CrossRefPubMed
23.
go back to reference Oikonomou A, Manavis J, Karagianni P, Tsanakas J, Wells AU, Hansell DM et al (2002) Loss of FEV1 in cystic fibrosis: correlation with HRCT features. European Radiology 12:2229–35CrossRefPubMed Oikonomou A, Manavis J, Karagianni P, Tsanakas J, Wells AU, Hansell DM et al (2002) Loss of FEV1 in cystic fibrosis: correlation with HRCT features. European Radiology 12:2229–35CrossRefPubMed
24.
go back to reference Paulin GA, Svenningsen S, Jobse BN, Mohan S, Kirby M, Lewis JF et al (2015) Differences in hyperpolarized (3) He ventilation imaging after 4 years in adults with cystic fibrosis. Journal of Magnetic Resonance Imaging 41:1701–7CrossRefPubMed Paulin GA, Svenningsen S, Jobse BN, Mohan S, Kirby M, Lewis JF et al (2015) Differences in hyperpolarized (3) He ventilation imaging after 4 years in adults with cystic fibrosis. Journal of Magnetic Resonance Imaging 41:1701–7CrossRefPubMed
25.
go back to reference Rosenbluth DB, Wilson K, Ferkol T, Schuster DP (2004) Lung function decline in cystic fibrosis patients and timing for lung transplantation referral. Chest 126:412–9CrossRefPubMed Rosenbluth DB, Wilson K, Ferkol T, Schuster DP (2004) Lung function decline in cystic fibrosis patients and timing for lung transplantation referral. Chest 126:412–9CrossRefPubMed
26.
go back to reference Com G, Carroll JL, Castro MM, Tang X, Jambhekar S, Berlinski A (2014) Predictors and outcome of low initial forced expiratory volume in 1 second measurement in children with cystic fibrosis. The Journal of Pediatrics 164:832–8CrossRefPubMed Com G, Carroll JL, Castro MM, Tang X, Jambhekar S, Berlinski A (2014) Predictors and outcome of low initial forced expiratory volume in 1 second measurement in children with cystic fibrosis. The Journal of Pediatrics 164:832–8CrossRefPubMed
27.
go back to reference Logan PM, O'Laoide RM, Mulherin D, O'Mahony S, FitzGerald MX, Masterson JB (1996) High resolution computed tomography in cystic fibrosis: correlation with pulmonary function and assessment of prognostic value. Irish Journal of Medical Science 165:27–31CrossRefPubMed Logan PM, O'Laoide RM, Mulherin D, O'Mahony S, FitzGerald MX, Masterson JB (1996) High resolution computed tomography in cystic fibrosis: correlation with pulmonary function and assessment of prognostic value. Irish Journal of Medical Science 165:27–31CrossRefPubMed
28.
go back to reference Sanders DB, Li Z, Brody AS (2015) Chest computed tomography predicts the frequency of pulmonary exacerbations in children with cystic fibrosis. Annals of the American Thoracic Society 12:64–9CrossRefPubMedPubMedCentral Sanders DB, Li Z, Brody AS (2015) Chest computed tomography predicts the frequency of pulmonary exacerbations in children with cystic fibrosis. Annals of the American Thoracic Society 12:64–9CrossRefPubMedPubMedCentral
29.
go back to reference McEvoy S, Lavelle L, Kilcoyne A, McCarthy C, deJong PA, Loeve M et al (2012) High-resolution CT of nontuberculous mycobacterium infection in adult CF patients: diagnostic accuracy. European Radiology 22:2736–42CrossRefPubMed McEvoy S, Lavelle L, Kilcoyne A, McCarthy C, deJong PA, Loeve M et al (2012) High-resolution CT of nontuberculous mycobacterium infection in adult CF patients: diagnostic accuracy. European Radiology 22:2736–42CrossRefPubMed
30.
go back to reference Desai SR, Edey AJ, Hansell DM, Shah A, Land D, Arnott S et al (2014) Morphologic predictors of a microbiological yield in patients with a tree-in-bud pattern on computed tomography. Journal of Thoracic Imaging 29:240–5CrossRefPubMed Desai SR, Edey AJ, Hansell DM, Shah A, Land D, Arnott S et al (2014) Morphologic predictors of a microbiological yield in patients with a tree-in-bud pattern on computed tomography. Journal of Thoracic Imaging 29:240–5CrossRefPubMed
31.
go back to reference Okada F, Ando Y, Yoshitake S, Ono A, Tanoue S, Matsumoto S et al (2007) Clinical/pathologic correlations in 553 patients with primary centrilobular findings on high-resolution CT scan of the thorax. Chest 132:1939–48CrossRefPubMed Okada F, Ando Y, Yoshitake S, Ono A, Tanoue S, Matsumoto S et al (2007) Clinical/pathologic correlations in 553 patients with primary centrilobular findings on high-resolution CT scan of the thorax. Chest 132:1939–48CrossRefPubMed
Metadata
Title
A semiquantitative MRI-Score can predict loss of lung function in patients with cystic fibrosis: Preliminary results
Authors
Juergen F. Schaefer
Andreas Hector
Katharina Schmidt
Matthias Teufel
Sabrina Fleischer
Ute Graepler-Mainka
Joachim Riethmueller
Sergios Gatidis
Susanne Schaefer
Konstantin Nikolaou
Dominik Hartl
Ilias Tsiflikas
Publication date
01-01-2018
Publisher
Springer Berlin Heidelberg
Published in
European Radiology / Issue 1/2018
Print ISSN: 0938-7994
Electronic ISSN: 1432-1084
DOI
https://doi.org/10.1007/s00330-017-4870-4

Other articles of this Issue 1/2018

European Radiology 1/2018 Go to the issue