Skip to main content
Top
Published in: BMC Pulmonary Medicine 1/2018

Open Access 01-12-2018 | Research article

Automated computed tomographic scoring of lung disease in adults with primary ciliary dyskinesia

Authors: Trieu-Nghi Hoang-Thi, Marie-Pierre Revel, Pierre-Régis Burgel, Laurence Bassinet, Isabelle Honoré, Thong Hua-Huy, Charlotte Martin, Bernard Maitre, Guillaume Chassagnon

Published in: BMC Pulmonary Medicine | Issue 1/2018

Login to get access

Abstract

Background

The present study aimed to develop an automated computed tomography (CT) score based on the CT quantification of high-attenuating lung structures, in order to provide a quantitative assessment of lung structural abnormalities in patients with Primary Ciliary Dyskinesia (PCD).

Methods

Adult (≥18 years) PCD patients who underwent both chest CT and spirometry within a 6-month period were retrospectively included. Commercially available lung segmentation software was used to isolate the lungs from the mediastinum and chest wall and obtain histograms of lung density. CT-density scores were calculated using fixed and adapted thresholds based on various combinations of histogram characteristics, such as mean lung density (MLD), skewness, and standard deviation (SD). Additionally, visual scoring using the Bhalla score was performed by 2 independent radiologists. Correlations between CT scores, forced expiratory volume in 1 s (FEV1) and forced vital capacity (FVC) were evaluated.

Results

Sixty-two adult patients with PCD were included. Of all histogram characteristics, those showing good positive or negative correlations to both FEV1 and FVC were SD (R = − 0.63 and − 0.67; p < 0.001) and Skewness (R = 0.67 and 0.67; p < 0.001). Among all evaluated thresholds, the CT-density score based on MLD + 1SD provided the best negative correlation with both FEV1 (R = − 0.68; p < 0.001) and FVC (R = − 0.71; p < 0.001), close to the correlations of the visual score (R = − 0.60; p < 0.001 for FEV1 and R = − 0.62; p < 0.001, for FVC).

Conclusions

Automated CT scoring of lung structural abnormalities lung in primary ciliary dyskinesia is feasible and may prove useful for evaluation of disease severity in the clinic and in clinical trials.
Literature
1.
go back to reference Afzelius BA. A human syndrome caused by immotile cilia. Science. 1976;193:317–9.CrossRef Afzelius BA. A human syndrome caused by immotile cilia. Science. 1976;193:317–9.CrossRef
2.
go back to reference Meeks M, Bush A. Primary ciliary dyskinesia (PCD). Pediatr Pulmonol. 2000;29:307–16.CrossRef Meeks M, Bush A. Primary ciliary dyskinesia (PCD). Pediatr Pulmonol. 2000;29:307–16.CrossRef
3.
go back to reference Honoré I, Burgel P-R. Primary ciliary dyskinesia in adults. Rev Mal Respir. 2016;33:165–89.CrossRef Honoré I, Burgel P-R. Primary ciliary dyskinesia in adults. Rev Mal Respir. 2016;33:165–89.CrossRef
4.
go back to reference Barbato A, Frischer T, Kuehni CE, Snijders D, Azevedo I, Baktai G, et al. Primary ciliary dyskinesia: a consensus statement on diagnostic and treatment approaches in children. Eur Respir J. 2009;34:1264–76.CrossRef Barbato A, Frischer T, Kuehni CE, Snijders D, Azevedo I, Baktai G, et al. Primary ciliary dyskinesia: a consensus statement on diagnostic and treatment approaches in children. Eur Respir J. 2009;34:1264–76.CrossRef
5.
go back to reference Shah A, Shoemark A, MacNeill SJ, Bhaludin B, Rogers A, Bilton D, et al. A longitudinal study characterising a large adult primary ciliary dyskinesia population. Eur Respir J. 2016;48:441–50.CrossRef Shah A, Shoemark A, MacNeill SJ, Bhaludin B, Rogers A, Bilton D, et al. A longitudinal study characterising a large adult primary ciliary dyskinesia population. Eur Respir J. 2016;48:441–50.CrossRef
6.
go back to reference Kennedy MP, Noone PG, Leigh MW, Zariwala MA, Minnix SL, Knowles MR, et al. High-resolution CT of patients with primary ciliary dyskinesia. AJR Am J Roentgenol. 2007;188:1232–8.CrossRef Kennedy MP, Noone PG, Leigh MW, Zariwala MA, Minnix SL, Knowles MR, et al. High-resolution CT of patients with primary ciliary dyskinesia. AJR Am J Roentgenol. 2007;188:1232–8.CrossRef
7.
go back to reference Santamaria F, Montella S, Tiddens HAWM, Guidi G, Casotti V, Maglione M, et al. Structural and functional lung disease in primary ciliary dyskinesia. Chest. 2008;134:351–7.CrossRef Santamaria F, Montella S, Tiddens HAWM, Guidi G, Casotti V, Maglione M, et al. Structural and functional lung disease in primary ciliary dyskinesia. Chest. 2008;134:351–7.CrossRef
8.
go back to reference Maglione M, Bush A, Montella S, Mollica C, Manna A, Esposito A, et al. Progression of lung disease in primary ciliary dyskinesia: is spirometry less accurate than CT? Pediatr Pulmonol. 2012;47:498–504.CrossRef Maglione M, Bush A, Montella S, Mollica C, Manna A, Esposito A, et al. Progression of lung disease in primary ciliary dyskinesia: is spirometry less accurate than CT? Pediatr Pulmonol. 2012;47:498–504.CrossRef
9.
go back to reference Magnin ML, Cros P, Beydon N, Mahloul M, Tamalet A, Escudier E, et al. Longitudinal lung function and structural changes in children with primary ciliary dyskinesia. Pediatr Pulmonol. 2012;47:816–25.CrossRef Magnin ML, Cros P, Beydon N, Mahloul M, Tamalet A, Escudier E, et al. Longitudinal lung function and structural changes in children with primary ciliary dyskinesia. Pediatr Pulmonol. 2012;47:816–25.CrossRef
10.
go back to reference Pifferi M, Bush A, Pioggia G, Caramella D, Tartarisco G, Di Cicco M, et al. Evaluation of pulmonary disease using static lung volumes in primary ciliary dyskinesia. Thorax. 2012;67:993–9.CrossRef Pifferi M, Bush A, Pioggia G, Caramella D, Tartarisco G, Di Cicco M, et al. Evaluation of pulmonary disease using static lung volumes in primary ciliary dyskinesia. Thorax. 2012;67:993–9.CrossRef
11.
go back to reference Cohen-Cymberknoh M, Simanovsky N, Hiller N, Gileles Hillel A, Shoseyov D, Kerem E. Differences in disease expression between primary ciliary dyskinesia and cystic fibrosis with and without pancreatic insufficiency. Chest. 2014;145:738–44.CrossRef Cohen-Cymberknoh M, Simanovsky N, Hiller N, Gileles Hillel A, Shoseyov D, Kerem E. Differences in disease expression between primary ciliary dyskinesia and cystic fibrosis with and without pancreatic insufficiency. Chest. 2014;145:738–44.CrossRef
12.
go back to reference Boon M, Vermeulen FL, Gysemans W, Proesmans M, Jorissen M, De Boeck K. Lung structure-function correlation in patients with primary ciliary dyskinesia. Thorax. 2015;70:339–45.CrossRef Boon M, Vermeulen FL, Gysemans W, Proesmans M, Jorissen M, De Boeck K. Lung structure-function correlation in patients with primary ciliary dyskinesia. Thorax. 2015;70:339–45.CrossRef
13.
go back to reference Frija-Masson J, Bassinet L, Honoré I, Dufeu N, Housset B, Coste A, et al. Clinical characteristics, functional respiratory decline and follow-up in adult patients with primary ciliary dyskinesia. Thorax. 2017;72:154–60.CrossRef Frija-Masson J, Bassinet L, Honoré I, Dufeu N, Housset B, Coste A, et al. Clinical characteristics, functional respiratory decline and follow-up in adult patients with primary ciliary dyskinesia. Thorax. 2017;72:154–60.CrossRef
14.
go back to reference Kennedy MP, Omran H, Leigh MW, Dell S, Morgan L, Molina PL, et al. Congenital heart disease and other heterotaxic defects in a large cohort of patients with primary ciliary dyskinesia. Circulation. 2007;115:2814–21.CrossRef Kennedy MP, Omran H, Leigh MW, Dell S, Morgan L, Molina PL, et al. Congenital heart disease and other heterotaxic defects in a large cohort of patients with primary ciliary dyskinesia. Circulation. 2007;115:2814–21.CrossRef
15.
go back to reference Calder AD, Bush A, Brody AS, Owens CM. Scoring of chest CT in children with cystic fibrosis: state of the art. Pediatr Radiol. 2014;44:1496–506.CrossRef Calder AD, Bush A, Brody AS, Owens CM. Scoring of chest CT in children with cystic fibrosis: state of the art. Pediatr Radiol. 2014;44:1496–506.CrossRef
16.
go back to reference Gevenois PA, de Maertelaer V, De Vuyst P, Zanen J, Yernault JC. Comparison of computed density and macroscopic morphometry in pulmonary emphysema. Am J Respir Crit Care Med. 1995;152:653–7.CrossRef Gevenois PA, de Maertelaer V, De Vuyst P, Zanen J, Yernault JC. Comparison of computed density and macroscopic morphometry in pulmonary emphysema. Am J Respir Crit Care Med. 1995;152:653–7.CrossRef
17.
go back to reference Chassagnon G, Martin C, Burgel P-R, Hubert D, Fajac I, Paragios N, et al. An automated computed tomography score for the cystic fibrosis lung. Eur Radiol. 2018. Chassagnon G, Martin C, Burgel P-R, Hubert D, Fajac I, Paragios N, et al. An automated computed tomography score for the cystic fibrosis lung. Eur Radiol. 2018.
18.
go back to reference Lucas JS, Barbato A, Collins SA, Goutaki M, Behan L, Caudri D, et al. European Respiratory Society guidelines for the diagnosis of primary ciliary dyskinesia. Eur Respir J. 2017;49:1601090. Lucas JS, Barbato A, Collins SA, Goutaki M, Behan L, Caudri D, et al. European Respiratory Society guidelines for the diagnosis of primary ciliary dyskinesia. Eur Respir J. 2017;49:1601090.
19.
go back to reference Chassagnon G, Morel B, Carpentier E, Ducou Le Pointe H, Sirinelli D. Tracheobronchial branching abnormalities: lobe-based classification scheme. Radiographics. 2016;36:358-73. Chassagnon G, Morel B, Carpentier E, Ducou Le Pointe H, Sirinelli D. Tracheobronchial branching abnormalities: lobe-based classification scheme. Radiographics. 2016;36:358-73.
20.
go back to reference Bhalla M, Turcios N, Aponte V, Jenkins M, Leitman BS, McCauley DI, et al. Cystic fibrosis: scoring system with thin-section CT. Radiology. 1991;179:783–8.CrossRef Bhalla M, Turcios N, Aponte V, Jenkins M, Leitman BS, McCauley DI, et al. Cystic fibrosis: scoring system with thin-section CT. Radiology. 1991;179:783–8.CrossRef
21.
go back to reference Goris ML, Zhu HJ, Blankenberg F, Chan F, Robinson TE. An automated approach to quantitative air trapping measurements in mild cystic fibrosis. Chest. 2003;123:1655–63.CrossRef Goris ML, Zhu HJ, Blankenberg F, Chan F, Robinson TE. An automated approach to quantitative air trapping measurements in mild cystic fibrosis. Chest. 2003;123:1655–63.CrossRef
22.
go back to reference Goris ML, Zhu HJ. Robinson TE. A critical discussion of computer analysis in medical imaging. Proc Am Thorac Soc. 2007;4:347–9.CrossRef Goris ML, Zhu HJ. Robinson TE. A critical discussion of computer analysis in medical imaging. Proc Am Thorac Soc. 2007;4:347–9.CrossRef
23.
go back to reference Miller MR, Hankinson J, Brusasco V, Burgos F, Casaburi R, Coates A, et al. Standardisation of spirometry. Eur Respir J. 2005;26:319–38.CrossRef Miller MR, Hankinson J, Brusasco V, Burgos F, Casaburi R, Coates A, et al. Standardisation of spirometry. Eur Respir J. 2005;26:319–38.CrossRef
24.
go back to reference Quanjer PH, Tammeling GJ, Cotes JE, Pedersen OF, Peslin R, Yernault JC. Lung volumes and forced ventilatory flows. Report working party standardization of lung function tests, European Community for steel and coal. Official statement of the European Respiratory Society. Eur Respir J Suppl. 1993;16:5–40.CrossRef Quanjer PH, Tammeling GJ, Cotes JE, Pedersen OF, Peslin R, Yernault JC. Lung volumes and forced ventilatory flows. Report working party standardization of lung function tests, European Community for steel and coal. Official statement of the European Respiratory Society. Eur Respir J Suppl. 1993;16:5–40.CrossRef
25.
go back to reference Robinson PJ, Kreel L. Pulmonary tissue attenuation with computed tomography: comparison of inspiration and expiration scans. J Comput Assist Tomogr. 1979;3:740–8.CrossRef Robinson PJ, Kreel L. Pulmonary tissue attenuation with computed tomography: comparison of inspiration and expiration scans. J Comput Assist Tomogr. 1979;3:740–8.CrossRef
26.
go back to reference Levi C, Gray JE, McCullough EC, Hattery RR. The unreliability of CT numbers as absolute values. AJR Am J Roentgenol. 1982;139:443–7.CrossRef Levi C, Gray JE, McCullough EC, Hattery RR. The unreliability of CT numbers as absolute values. AJR Am J Roentgenol. 1982;139:443–7.CrossRef
27.
go back to reference Rudyanto RD, Kerkstra S, van Rikxoort EM, Fetita C, Brillet P-Y, Lefevre C, et al. Comparing algorithms for automated vessel segmentation in computed tomography scans of the lung: the VESSEL12 study. Med Image Anal. 2014;18:1217–32.CrossRef Rudyanto RD, Kerkstra S, van Rikxoort EM, Fetita C, Brillet P-Y, Lefevre C, et al. Comparing algorithms for automated vessel segmentation in computed tomography scans of the lung: the VESSEL12 study. Med Image Anal. 2014;18:1217–32.CrossRef
28.
go back to reference Loeve M, Krestin GP, Rosenfeld M, de Bruijne M, Stick SM, Tiddens HA. Chest computed tomography: a validated surrogate endpoint of cystic fibrosis lung disease? Eur Respir J. 2013;42:844–57.CrossRef Loeve M, Krestin GP, Rosenfeld M, de Bruijne M, Stick SM, Tiddens HA. Chest computed tomography: a validated surrogate endpoint of cystic fibrosis lung disease? Eur Respir J. 2013;42:844–57.CrossRef
Metadata
Title
Automated computed tomographic scoring of lung disease in adults with primary ciliary dyskinesia
Authors
Trieu-Nghi Hoang-Thi
Marie-Pierre Revel
Pierre-Régis Burgel
Laurence Bassinet
Isabelle Honoré
Thong Hua-Huy
Charlotte Martin
Bernard Maitre
Guillaume Chassagnon
Publication date
01-12-2018
Publisher
BioMed Central
Published in
BMC Pulmonary Medicine / Issue 1/2018
Electronic ISSN: 1471-2466
DOI
https://doi.org/10.1186/s12890-018-0758-6

Other articles of this Issue 1/2018

BMC Pulmonary Medicine 1/2018 Go to the issue