Korean J Radiol.  2017 Feb;18(1):260-267. 10.3348/kjr.2017.18.1.260.

Chest CT Features of Cystic Fibrosis in Korea: Comparison with Non-Cystic Fibrosis Diseases

• Yang SY
• Lee KS
• Cha MJ
• Kim TJ
• Kim TS
• Yoon HJ

• KMID: 2468138
• DOI: http://doi.org/10.3348/kjr.2017.18.1.260

Abstract

OBJECTIVE
Cystic fibrosis (CF) is a rare congenital disease in Korea, and its clinical and imaging findings are unclear. The objective of our study was to describe the clinical and CT features of CF in Korea and compare its features with those of other diseases mimicking CF.
MATERIALS AND METHODS
From November 1994 to December 2014, a presumptive diagnosis of CF was made in 23 patients based on clinical or radiological examination. After the exclusion of 10 patients without diagnostic confirmation, 13 patients were included in the study. A diagnosis of CF was made with the CF gene study. CT findings were evaluated for the presence and distribution of parenchymal abnormalities including bronchiectasis, tree-in-bud (TIB) pattern, mucus plugging, consolidation, and mosaic attenuation.
RESULTS
Of the 13 patients, 7 (median age, 15 years) were confirmed as CF, 4 (median age, 19 years) had primary ciliary dyskinesia, 1 had bronchiectasis of unknown cause, and 1 had chronic asthma. CT of patients with CF showed bilateral bronchiectasis, TIB pattern, mosaic attenuation, and mucus plugging in all patients, with upper lung predominance (57%). In CT of the non-CF patients, bilateral bronchiectasis, TIB pattern, mosaic attenuation, and mucus plugging were also predominant features, with lower lung predominance (50%).
CONCLUSION
Korean patients with CF showed bilateral bronchiectasis, cellular bronchiolitis, mucus plugging, and mosaic attenuation, which overlapped with those of non-CF patients. CF gene study is recommended for the definitive diagnosis of CF in patients with these clinical and imaging features.

Keyword

Cystic fibrosis; Computed tomography; Primary ciliary dyskinesia; Asthma; Korea

MeSH Terms

Adolescent
Adult
Asthma/diagnosis/diagnostic imaging
Bronchiectasis/diagnosis/diagnostic imaging
Child
Ciliary Motility Disorders/diagnosis/diagnostic imaging
Cystic Fibrosis/*diagnosis/diagnostic imaging
Female
Humans
Male
Republic of Korea
Tomography, X-Ray Computed
Young Adult

Figure

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  Fig. 1 Flowchart of study population. CF = cystic fibrosis, CFTR = cystic fibrosis transmembrane conductance regulator, EM = electromicroscopic, PCD = primary ciliary dyskinesia

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  Fig. 2 Cystic fibrosis in 15-year-old male patient who underwent lung transplantation. Lung window image of thin-section (2.5-mm-section thickness) CT scan obtained at levels of aortic arch shows extensive areas of bronchiectasis (arrows) and cellular bronchiolitis (arrowheads) in both lungs. Also note patchy areas (open arrows) of mosaic attenuation.

• 

  Fig. 3 17-year-old female patient with cystic fibrosis and Pseudomonas aeruginosa infection. A. Lung window image of thin-section (2.0-mm-section thickness) CT obtained at level of aortic arch shows extensive areas of bronchiectasis and cellular bronchiolitis (arrowheads) in both lungs. Also note wide areas (open arrows) of mosaic attenuation. Several areas of rectangular consolidation (thin arrows) suggest presence of concurrent bacterial pneumonia. B. Coronal reformatted (2.0-mm-section thickness) CT image demonstrates areas of bronchiectasis (arrows), mosaic attenuation (open arrows), and mucus plugging (arrowheads) predominantly involving bilateral upper lung zones. Also note areas of parenchymal opacity (thin arrows).

• 

  Fig. 4 19-year-old female patient with primary ciliary dyskinesia syndrome and concurrent infection with Mycobacterium gordonae. A. Lung window image of thin-section (2.5-mm-section thickness) CT obtained at level of lower esophagus shows extensive areas of bronchiectasis (arrows) and mucus plugging (arrowheads) in both lungs. Also note patchy areas (open arrows) of mosaic attenuation. B. Coronal reformatted (2.0-mm-section thickness) CT image demonstrates bronchiectasis (arrows) predominantly in lower lung zones. Also note mucus plugging (arrowhead) and parenchymal consolidation (thin arrows).

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