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Published in: Insights into Imaging 6/2013

Open Access 01-12-2013 | Review

Radiography, tomosynthesis, CT and MRI in the evaluation of pulmonary cystic fibrosis: an untangling review of the multitude of scoring systems

Authors: Kristina Vult von Steyern, Isabella M. Björkman-Burtscher, Mats Geijer

Published in: Insights into Imaging | Issue 6/2013

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Abstract

Objective

The first radiographic scoring system for pulmonary cystic fibrosis was presented in 1958. Since then a multitude of scoring systems for radiography and computed tomography (CT) have been presented, recently also for tomosynthesis and magnetic resonance imaging (MRI). The aim of the current review was to analyse and compare the plethora of scoring systems for cystic fibrosis, especially regarding which scoring components are considered most important.

Methods

Four scoring systems for chest radiography, one for tomosynthesis, eight for CT and one for MRI were compared regarding components evaluated and their terminology; the areas scored; scoring levels; the weighting of each component in percentage of the total score; and the calculations for the final score.

Results

In most radiological scoring systems the lungs are evaluated for increased volume, bronchial wall thickening, bronchiectasis, mucus plugging, atelectasis and consolidation. In addition, for instance abscesses, bullae, septal thickening, mosaic perfusion, ground glass opacities and air trapping are evaluated in some CT scoring systems. Pleural affection and perfusion defects are scored on MRI.

Conclusions

Bronchiectasis alone, or in combination with mucus plugging, is given the highest weighting in most scoring systems and is thus commonly considered to be the most significant finding when evaluating cystic fibrosis lung disease.

Teaching points

  • Scoring of examinations is used for comparison of outcome in studies.
  • Scoring of examinations can also be used for monitoring disease progression.
  • Cystic fibrosis can be scored on radiography, tomosynthesis, CT or MRI.
  • The typical imaging findings of cystic fibrosis depend on the imaging modality used.
  • Bronchiectasis is commonly considered the most significant finding when scoring cystic fibrosis.
Literature
1.
2.
go back to reference Cooper P, MacLean J (2006) High-resolution computed tomography (HRCT) should not be considered as a routine assessment method in cystic fibrosis lung disease. Paediatr Respir Rev 7:197–201PubMedCrossRef Cooper P, MacLean J (2006) High-resolution computed tomography (HRCT) should not be considered as a routine assessment method in cystic fibrosis lung disease. Paediatr Respir Rev 7:197–201PubMedCrossRef
3.
go back to reference Tiddens H (2006) Chest computed tomography scans should be considered as a routine investigation in cystic fibrosis. Paediatr Respir Rev 7:202–208PubMedCrossRef Tiddens H (2006) Chest computed tomography scans should be considered as a routine investigation in cystic fibrosis. Paediatr Respir Rev 7:202–208PubMedCrossRef
4.
go back to reference Vult von Steyern K, Björkman-Burtscher I, Geijer M (2012) Tomosynthesis in pulmonary cystic fibrosis with comparison to radiography and computed tomography: a pictorial review. Insights Imaging 3:81–89PubMedPubMedCentralCrossRef Vult von Steyern K, Björkman-Burtscher I, Geijer M (2012) Tomosynthesis in pulmonary cystic fibrosis with comparison to radiography and computed tomography: a pictorial review. Insights Imaging 3:81–89PubMedPubMedCentralCrossRef
5.
go back to reference Vult von Steyern K, Björkman-Burtscher IM, Höglund P, Bozovic G, Wiklund M, Geijer M (2012) Description and validation of a scoring system for tomosynthesis in pulmonary cystic fibrosis. Eur Radiol 22:2718–2728PubMedCrossRef Vult von Steyern K, Björkman-Burtscher IM, Höglund P, Bozovic G, Wiklund M, Geijer M (2012) Description and validation of a scoring system for tomosynthesis in pulmonary cystic fibrosis. Eur Radiol 22:2718–2728PubMedCrossRef
6.
go back to reference Båth M, Svalkvist A, von Wrangel A, Rismyhr-Olsson H, Cederblad Å (2010) Effective dose to patients from chest examinations with tomosynthesis. Radiat Prot Dosim 139:153–158CrossRef Båth M, Svalkvist A, von Wrangel A, Rismyhr-Olsson H, Cederblad Å (2010) Effective dose to patients from chest examinations with tomosynthesis. Radiat Prot Dosim 139:153–158CrossRef
7.
go back to reference Eichinger M, Puderbach M, Fink C et al (2006) Contrast-enhanced 3D MRI of lung perfusion in children with cystic fibrosis-initial results. Eur Radiol 16:2147–2152PubMedCrossRef Eichinger M, Puderbach M, Fink C et al (2006) Contrast-enhanced 3D MRI of lung perfusion in children with cystic fibrosis-initial results. Eur Radiol 16:2147–2152PubMedCrossRef
8.
go back to reference Puderbach M, Eichinger M, Gahr J et al (2007) Proton MRI appearance of cystic fibrosis: comparison to CT. Eur Radiol 17:716–724PubMedCrossRef Puderbach M, Eichinger M, Gahr J et al (2007) Proton MRI appearance of cystic fibrosis: comparison to CT. Eur Radiol 17:716–724PubMedCrossRef
9.
go back to reference Eichinger M, Optazaite D-E, Kopp-Schneider A et al (2012) Morphologic and functional scoring of cystic fibrosis lung disease using MRI. Eur J Radiol 81:1321–1329PubMedCrossRef Eichinger M, Optazaite D-E, Kopp-Schneider A et al (2012) Morphologic and functional scoring of cystic fibrosis lung disease using MRI. Eur J Radiol 81:1321–1329PubMedCrossRef
10.
go back to reference Shwachman H, Kulczycki L (1958) Long-term study of one hundred five patients with cystic fibrosis: studies made over a five-to fourteen-year period. AMA J Dis Child 96:6–15PubMedCrossRef Shwachman H, Kulczycki L (1958) Long-term study of one hundred five patients with cystic fibrosis: studies made over a five-to fourteen-year period. AMA J Dis Child 96:6–15PubMedCrossRef
11.
go back to reference Brasfield D, Hicks G, Soong S, Tiller RE (1979) The chest roentgenogram in cystic fibrosis: a new scoring system. Pediatrics 63:24–29PubMed Brasfield D, Hicks G, Soong S, Tiller RE (1979) The chest roentgenogram in cystic fibrosis: a new scoring system. Pediatrics 63:24–29PubMed
12.
go back to reference Taussig L, Kattwinkel J, Friedewald W, Di Sant’Agnese PA (1973) A new prognostic score and clinical evaluation system for cystic fibrosis. J Pediatr 82:380–390PubMedCrossRef Taussig L, Kattwinkel J, Friedewald W, Di Sant’Agnese PA (1973) A new prognostic score and clinical evaluation system for cystic fibrosis. J Pediatr 82:380–390PubMedCrossRef
13.
go back to reference Chrispin A, Norman A (1974) The systematic evaluation of the chest radiograph in cystic fibrosis. Pediatr Radiol 2:101–105PubMedCrossRef Chrispin A, Norman A (1974) The systematic evaluation of the chest radiograph in cystic fibrosis. Pediatr Radiol 2:101–105PubMedCrossRef
15.
go back to reference Van der Put J, Meradji M, Danoesastro D, Kerrebijn KF (1982) Chest radiographs in cystic fibrosis. A follow-up study with application of a quantitative system. Pediatr Radiol 12:57–61PubMedCrossRef Van der Put J, Meradji M, Danoesastro D, Kerrebijn KF (1982) Chest radiographs in cystic fibrosis. A follow-up study with application of a quantitative system. Pediatr Radiol 12:57–61PubMedCrossRef
16.
go back to reference Carty H (1987) The chest radiograph in cystic fibrosis in children and the role of other radiological techniques. J R Soc Med 80:38–46PubMedPubMedCentral Carty H (1987) The chest radiograph in cystic fibrosis in children and the role of other radiological techniques. J R Soc Med 80:38–46PubMedPubMedCentral
17.
go back to reference O’Laoide R, Fahy J, Coffey M et al (1991) A chest radiograph scoring system in adult cystic fibrosis: Correlation with pulmonary function. Clin Radiol 43:308–310PubMedCrossRef O’Laoide R, Fahy J, Coffey M et al (1991) A chest radiograph scoring system in adult cystic fibrosis: Correlation with pulmonary function. Clin Radiol 43:308–310PubMedCrossRef
18.
go back to reference Weatherly M, Palmer C, Peters M et al (1993) Wisconsin cystic fibrosis chest radiograph scoring system. Pediatrics 91:488–495PubMed Weatherly M, Palmer C, Peters M et al (1993) Wisconsin cystic fibrosis chest radiograph scoring system. Pediatrics 91:488–495PubMed
19.
go back to reference Conway S, Pond M, Bowler I et al (1994) The chest radiograph in cystic fibrosis: a new scoring system compared with the Chrispin-Norman and Brasfield scores. Thorax 49:860–862PubMedPubMedCentralCrossRef Conway S, Pond M, Bowler I et al (1994) The chest radiograph in cystic fibrosis: a new scoring system compared with the Chrispin-Norman and Brasfield scores. Thorax 49:860–862PubMedPubMedCentralCrossRef
20.
go back to reference Koscik R, Kosorok M, Farrell P et al (2000) Wisconsin cystic fibrosis chest radiograph scoring system: validation and standardization for application to longitudinal studies. Pediatr Pulmonol 29:457–467PubMedCrossRef Koscik R, Kosorok M, Farrell P et al (2000) Wisconsin cystic fibrosis chest radiograph scoring system: validation and standardization for application to longitudinal studies. Pediatr Pulmonol 29:457–467PubMedCrossRef
21.
go back to reference Benden C, Wallis C, Owens C, Ridout DA, Dinwiddie R (2005) The Chrispin-Norman score in cystic fibrosis: doing away with the lateral view. Eur Respir J 26:894–897PubMedCrossRef Benden C, Wallis C, Owens C, Ridout DA, Dinwiddie R (2005) The Chrispin-Norman score in cystic fibrosis: doing away with the lateral view. Eur Respir J 26:894–897PubMedCrossRef
22.
go back to reference Bhalla M, Turcios N, Aponte V et al (1991) Cystic fibrosis: scoring system with thin-section CT. Radiology 179:783–788PubMedCrossRef Bhalla M, Turcios N, Aponte V et al (1991) Cystic fibrosis: scoring system with thin-section CT. Radiology 179:783–788PubMedCrossRef
23.
go back to reference Nathanson I, Conboy K, Murphy S, Afshani E, Kuhn JP (1991) Ultrafast computerized tomography of the chest in cystic fibrosis: a new scoring system. Pediatr Pulmonol 11:81–86PubMedCrossRef Nathanson I, Conboy K, Murphy S, Afshani E, Kuhn JP (1991) Ultrafast computerized tomography of the chest in cystic fibrosis: a new scoring system. Pediatr Pulmonol 11:81–86PubMedCrossRef
24.
go back to reference Stiglbauer R, Schurawitzki H, Eichler I, Vergesslich KA, Götz M (1992) High resolution CT in children with cystic fibrosis. Acta Radiol 33:548–553PubMedCrossRef Stiglbauer R, Schurawitzki H, Eichler I, Vergesslich KA, Götz M (1992) High resolution CT in children with cystic fibrosis. Acta Radiol 33:548–553PubMedCrossRef
25.
go back to reference Maffessanti M, Candusso M, Brizzi F, Piovesana F (1996) Cystic fibrosis in children: HRCT findings and distribution of disease. J Thorac Imaging 11:27–38PubMedCrossRef Maffessanti M, Candusso M, Brizzi F, Piovesana F (1996) Cystic fibrosis in children: HRCT findings and distribution of disease. J Thorac Imaging 11:27–38PubMedCrossRef
26.
go back to reference Donnelly L, Gelfand MJ, Brody AS, Wilmott RW (1997) Comparison between morphologic changes seen on high-resolution CT and regional pulmonary perfusion seen on SPECT in patients with cystic fibrosis. Pediatr Radiol 27:920–925PubMedCrossRef Donnelly L, Gelfand MJ, Brody AS, Wilmott RW (1997) Comparison between morphologic changes seen on high-resolution CT and regional pulmonary perfusion seen on SPECT in patients with cystic fibrosis. Pediatr Radiol 27:920–925PubMedCrossRef
27.
go back to reference Shah R, Sexauer W, Ostrum B, 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 Am J Roentgenol 169:375–380PubMedCrossRef Shah R, Sexauer W, Ostrum B, 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 Am J Roentgenol 169:375–380PubMedCrossRef
28.
go back to reference Santamaria F, Grillo G, Guidi G et al (1998) Cystic fibrosis: when should high-resolution computed tomography of the chest be obtained? Pediatrics 101:908–913PubMedCrossRef Santamaria F, Grillo G, Guidi G et al (1998) Cystic fibrosis: when should high-resolution computed tomography of the chest be obtained? Pediatrics 101:908–913PubMedCrossRef
29.
go back to reference Brody A, Molina P, Klein J, Rothman BS, Ramagopal M, Swartz DR (1999) High-resolution computed tomography of the chest in children with cystic fibrosis: support for use as an outcome surrogate. Pediatr Radiol 29:731–735PubMedCrossRef Brody A, Molina P, Klein J, Rothman BS, Ramagopal M, Swartz DR (1999) High-resolution computed tomography of the chest in children with cystic fibrosis: support for use as an outcome surrogate. Pediatr Radiol 29:731–735PubMedCrossRef
30.
go back to reference Helbich T, Heinz-Peer G, Eichler I et al (1999) Cystic fibrosis: CT assessment of lung involvement in children and adults. Radiology 213:537–544PubMedCrossRef Helbich T, Heinz-Peer G, Eichler I et al (1999) Cystic fibrosis: CT assessment of lung involvement in children and adults. Radiology 213:537–544PubMedCrossRef
31.
go back to reference Castile R, Hayes J, Flucke R, McCoy KS (2000) Correlation of structural and functional abnormalities in the lungs of infants with cystic fibrosis (Abstract). Pediatr Pulmonol 20:A427 Castile R, Hayes J, Flucke R, McCoy KS (2000) Correlation of structural and functional abnormalities in the lungs of infants with cystic fibrosis (Abstract). Pediatr Pulmonol 20:A427
32.
go back to reference Robinson T, Leung A, Northway W et al (2001) Spirometer-triggered high-resolution computed tomography and pulmonary function measurements during an acute exacerbation in patients with cystic fibrosis. J Pediatr 138:553–559PubMedCrossRef Robinson T, Leung A, Northway W et al (2001) Spirometer-triggered high-resolution computed tomography and pulmonary function measurements during an acute exacerbation in patients with cystic fibrosis. J Pediatr 138:553–559PubMedCrossRef
33.
go back to reference Oikonomou A, Manavis J, Karagianni P et al (2002) Loss of FEV1 in cystic fibrosis: correlation with HRCT features. Eur Radiol 12:2229–2235PubMedCrossRef Oikonomou A, Manavis J, Karagianni P et al (2002) Loss of FEV1 in cystic fibrosis: correlation with HRCT features. Eur Radiol 12:2229–2235PubMedCrossRef
34.
go back to reference Brody A, Klein J, Molina P, Quan J, Bean JA, Wilmott RW (2004) High-resolution computed tomography in young patients with cystic fibrosis: distribution of abnormalities and correlation with pulmonary function tests. J Pediatr 145:32–38PubMedCrossRef Brody A, Klein J, Molina P, Quan J, Bean JA, Wilmott RW (2004) High-resolution computed tomography in young patients with cystic fibrosis: distribution of abnormalities and correlation with pulmonary function tests. J Pediatr 145:32–38PubMedCrossRef
35.
go back to reference Oikonomou A, Tsanakas J, Hatziagorou E et al (2008) High resolution computed tomography of the chest in cystic fibrosis (CF): is simplification of scoring systems feasible? Eur Radiol 18:538–547PubMedCrossRef Oikonomou A, Tsanakas J, Hatziagorou E et al (2008) High resolution computed tomography of the chest in cystic fibrosis (CF): is simplification of scoring systems feasible? Eur Radiol 18:538–547PubMedCrossRef
36.
go back to reference Wong E, Regnis J, Shnier R, Bye PT, Stewart ME (1993) The relationship between tests of lung function and three chest radiological scoring systems in patients with cystic fibrosis. Australas Radiol 37:265–269PubMedCrossRef Wong E, Regnis J, Shnier R, Bye PT, Stewart ME (1993) The relationship between tests of lung function and three chest radiological scoring systems in patients with cystic fibrosis. Australas Radiol 37:265–269PubMedCrossRef
38.
go back to reference Helbich T, Heinz-Peer G, Fleischmann D et al (1999) Evolution of CT findings in patients with cystic fibrosis. AJR Am J Roentgenol 173:81–88PubMedCrossRef Helbich T, Heinz-Peer G, Fleischmann D et al (1999) Evolution of CT findings in patients with cystic fibrosis. AJR Am J Roentgenol 173:81–88PubMedCrossRef
39.
go back to reference de Jong P, Ottink M, Robben S et al (2004) Pulmonary disease assessment in cystic fibrosis: comparison of CT scoring systems and value of bronchial and arterial dimension measurements. Radiology 231:434–439PubMedCrossRef de Jong P, Ottink M, Robben S et al (2004) Pulmonary disease assessment in cystic fibrosis: comparison of CT scoring systems and value of bronchial and arterial dimension measurements. Radiology 231:434–439PubMedCrossRef
40.
go back to reference de Jong P, Tiddens H (2007) Cystic fibrosis specific computed tomography scoring. Proc Am Thorac Soc 4:338–342PubMedCrossRef de Jong P, Tiddens H (2007) Cystic fibrosis specific computed tomography scoring. Proc Am Thorac Soc 4:338–342PubMedCrossRef
41.
go back to reference De Jong P, Nakano Y, Lequin M et al (2004) Progressive damage on high resolution computed tomography despite stable lung function in cystic fibrosis. Eur Respir J 23:93–97PubMedCrossRef De Jong P, Nakano Y, Lequin M et al (2004) Progressive damage on high resolution computed tomography despite stable lung function in cystic fibrosis. Eur Respir J 23:93–97PubMedCrossRef
42.
go back to reference Bonnel AS, Song SM, Kesavarju K et al (2004) Quantitative air-trapping analysis in children with mild cystic fibrosis lung disease. Pediatr Pulmonol 38:396–405PubMedCrossRef Bonnel AS, Song SM, Kesavarju K et al (2004) Quantitative air-trapping analysis in children with mild cystic fibrosis lung disease. Pediatr Pulmonol 38:396–405PubMedCrossRef
43.
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. Pediatr Pulmonol 35:441–445PubMedCrossRef 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. Pediatr Pulmonol 35:441–445PubMedCrossRef
44.
go back to reference Kerem E, Conway S, Elborn S, Heijerman H (2005) Standards of care for patients with cystic fibrosis: a European consensus. J Cyst Fibros 4:7–26PubMedCrossRef Kerem E, Conway S, Elborn S, Heijerman H (2005) Standards of care for patients with cystic fibrosis: a European consensus. J Cyst Fibros 4:7–26PubMedCrossRef
45.
go back to reference Puderbach M, Eichinger M, Haeselbarth J et al (2007) Assessment of morphological MRI for pulmonary changes in cystic fibrosis (CF) patients: comparison to thin-section CT and chest X-ray. Invest Radiol 42:715–724PubMedCrossRef Puderbach M, Eichinger M, Haeselbarth J et al (2007) Assessment of morphological MRI for pulmonary changes in cystic fibrosis (CF) patients: comparison to thin-section CT and chest X-ray. Invest Radiol 42:715–724PubMedCrossRef
46.
go back to reference Smith-Bindman R, Lipson J, Marcus R et al (2009) Radiation dose assosiated with common computed tomography examinations and the associated lifetime attributable risk of cancer. Arch Intern Med 169:2078–2086PubMedPubMedCentralCrossRef Smith-Bindman R, Lipson J, Marcus R et al (2009) Radiation dose assosiated with common computed tomography examinations and the associated lifetime attributable risk of cancer. Arch Intern Med 169:2078–2086PubMedPubMedCentralCrossRef
47.
go back to reference Mettler FA, Huda W, Yoshizumi TT, Mahesh M (2008) Effective doses in radiology and diagnostic nuclear medicine: a catalog. Radiology 24:254–263CrossRef Mettler FA, Huda W, Yoshizumi TT, Mahesh M (2008) Effective doses in radiology and diagnostic nuclear medicine: a catalog. Radiology 24:254–263CrossRef
48.
go back to reference Loeve M, Lequin MH, de Bruijne M et al (2009) Cystic fibrosis: are volumetric ultra-low-dose expiratory CT scans sufficient for monitoring related lung disease? Radiology 253:223–229PubMedCrossRef Loeve M, Lequin MH, de Bruijne M et al (2009) Cystic fibrosis: are volumetric ultra-low-dose expiratory CT scans sufficient for monitoring related lung disease? Radiology 253:223–229PubMedCrossRef
Metadata
Title
Radiography, tomosynthesis, CT and MRI in the evaluation of pulmonary cystic fibrosis: an untangling review of the multitude of scoring systems
Authors
Kristina Vult von Steyern
Isabella M. Björkman-Burtscher
Mats Geijer
Publication date
01-12-2013
Publisher
Springer Berlin Heidelberg
Published in
Insights into Imaging / Issue 6/2013
Electronic ISSN: 1869-4101
DOI
https://doi.org/10.1007/s13244-013-0288-y

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