Structural and Functional Lung Impairment in PCD: Assessment with MRI and Multiple Breath Washout in Comparison to Spirometry.

Nyilas, Sylvia; Bauman, Grzegorz; Pusterla, Orso; Sommer, Gregor; Singer, Florian; Stranzinger, Enno; Heyer, Christoph; Ramsey, Kathryn Angela; Schlegtendal, Anne; Benzrath, Stefanie; Casaulta, Carmen; Goutaki, Myrofora; Kuehni, Claudia E; Bieri, Oliver; Koerner-Rettberg, Cordula; Latzin, Philipp (2018). Structural and Functional Lung Impairment in PCD: Assessment with MRI and Multiple Breath Washout in Comparison to Spirometry. Annals of the American Thoracic Society, 15(12), pp. 1434-1442. American Thoracic Society 10.1513/AnnalsATS.201712-967OC

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RATIONALE Primary ciliary dyskinesia (PCD) is an inherited disorder characterized by heterogeneous airway disease. Traditional lung function techniques (e.g. spirometry) may underestimate severity and complexity of PCD. OBJECTIVES We assessed lung impairment in individuals with PCD using structural and functional magnetic resonance imaging (MRI) and different lung function techniques. METHODS Thirty study participants with PCD (median 13.4 years, range 5-28) underwent structural and functional MRI, spirometry, and multiple breath washout (MBW) on the same day. Primary endpoints included structural MRI morphology scores, relative ventilation and perfusion impairment from functional MRI, FEV1 from spirometry, and lung clearance index (LCI) from MBW. RESULTS Severity and complexity of PCD lung disease varied significantly between individuals. Structural lung disease was detected in all subjects with a median (IQR) extent score of 10.3 (7 to 19; maximum score = 60). Functional MRI ventilation impairment was present in 52% of subjects affecting 24.2% (21.1 to 25.2%) of the lung. Relative perfusion impairment was detected in 78% of individuals affecting 21.1% (19.4 to 25.9%) of the lung. LCI was abnormal in 83% (median 8.3 (2.6 to 13.2) z-scores) and FEV1 was abnormal in 27% (-0.5 (-1.6 to 0.3) z-scores) of individuals. Concordance between spirometry and imaging outcomes was poor, with 52% of patients showing both abnormal MRI and LCI values, but normal FEV1. CONCLUSIONS Discordance between lung function and imaging outcomes in patients with PCD supports the use of both imaging and lung function, such as MBW, for surveillance of this heterogeneous disease.

Item Type:

Journal Article (Original Article)

Division/Institute:

04 Faculty of Medicine > Department of Gynaecology, Paediatrics and Endocrinology (DFKE) > Clinic of Paediatric Medicine
04 Faculty of Medicine > Pre-clinic Human Medicine > Institute of Social and Preventive Medicine
04 Faculty of Medicine > Department of Radiology, Neuroradiology and Nuclear Medicine (DRNN) > Institute of Diagnostic, Interventional and Paediatric Radiology

Graduate School:

Graduate School for Cellular and Biomedical Sciences (GCB)

UniBE Contributor:

Nyilas, Sylvia Meryl; Singer, Florian; Stranzinger, Enno; Ramsey, Kathryn Angela; Casaulta, Carmen; Goutaki, Myrofora; Kühni, Claudia and Latzin, Philipp

Subjects:

600 Technology > 610 Medicine & health
300 Social sciences, sociology & anthropology > 360 Social problems & social services

ISSN:

2329-6933

Publisher:

American Thoracic Society

Language:

English

Submitter:

Tanya Karrer

Date Deposited:

24 Oct 2018 15:18

Last Modified:

14 Jan 2019 14:39

Publisher DOI:

10.1513/AnnalsATS.201712-967OC

PubMed ID:

30290127

Additional Information:

Nyilas and Bauman contributed equally to this work. Koerner-Rettberg and Latzin contributed equally to this work.

BORIS DOI:

10.7892/boris.120536

URI:

https://boris.unibe.ch/id/eprint/120536

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