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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Nyilas AnnAmThoracSoc 2018_manuscript.pdf - Accepted Version Available under License Publisher holds Copyright. Download (1MB) | Preview |
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.
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