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Review
. 2018 May 22;18(1):79.
doi: 10.1186/s12890-018-0630-8.

Pathogenesis, imaging and clinical characteristics of CF and non-CF bronchiectasis

Jürgen Schäfer  1 , Matthias Griese  2 , Ravishankar Chandrasekaran  3 , Sanjay H Chotirmall  3 , Dominik Hartl  4   5
Affiliations
Review

Pathogenesis, imaging and clinical characteristics of CF and non-CF bronchiectasis

Jürgen Schäfer et al. BMC Pulm Med. .

Abstract

Bronchiectasis is a common feature of severe inherited and acquired pulmonary disease conditions. Among inherited diseases, cystic fibrosis (CF) is the major disorder associated with bronchiectasis, while acquired conditions frequently featuring bronchiectasis include post-infective bronchiectasis and chronic obstructive pulmonary disease (COPD). Mechanistically, bronchiectasis is driven by a complex interplay of inflammation and infection with neutrophilic inflammation playing a predominant role. The clinical characterization and management of bronchiectasis should involve a precise diagnostic workup, tailored therapeutic strategies and pulmonary imaging that has become an essential tool for the diagnosis and follow-up of bronchiectasis. Prospective future studies are required to optimize the diagnostic and therapeutic management of bronchiectasis, particularly in heterogeneous non-CF bronchiectasis populations.

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Conflict of interest statement

Ethics approval and consent to participate

Not applicable.

Consent for publication

Consent for publication has been obtained from all persons for the included images.

Competing interests

Sanjay Chotirmall is a member of the editorial board (Section Editor). Dominik Hartl has an affiliation with University of Tübingen and Roche Basel (I3-DTA, pRED). All other authors declare that they have no competing interests.

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Figures

Fig. 1
Fig. 1
Model of disease progress towards bronchiectasis in patients with and without CF. Modified from Chang et al. [115]
Fig. 2
Fig. 2
15 y/o male, CF-patient, FEV1 predicted 45%. Thin-section HR-reconstruction from MDCT (effective dose of 1.5 mSv). Severe bronchiectasis is visible. Note also dilated bronchi within the periphery of the lung. Air trapping is noted, only in central parenchyma, the CT attenuation appears normal (asterisks)
Fig. 3
Fig. 3
6 y/o female CF-patient, FEV1 predicted 105%. Thin-section HR-reconstruction from MDCT (effective dose of 1 mSv). Mild bronchiectasis, bronchial wall thickening (arrows) and mosaic attenuation (asterisk) are visible
Fig. 4
Fig. 4
29 y/o male CF-patient, FEV1 predicted 67%. Left side CT, right side MRI at the same day. Upper row, transverse thin-section images from 3D acquisition in breath hold (CT and MRI). Note, despite lower resolution and signal to noise a similar depiction of bronchiectasis is possible. Lower row, expiration images (transverse CT and saggital MRI). In both modalities focal air trapping within the same lung region is demonstrated (arrow heads)
Fig. 5
Fig. 5
Perfusion map of same patient as in fig. 4 using the noninvasive arterial-spin-labeling technique without contrast media application. The relevant perfusion differences between upper and lower lung regions correlate with morphological damage and air trapping
See this image and copyright information in PMC

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