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

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.

Deviations of body functions and structure, activity limitations, and participation restrictions of the International Classification of Functioning, Disability, and Health model in children with cystic fibrosis and non–cystic fibrosis bronchiectasis

“…In children diagnosed with cystic fibrosis (CF) or bronchiectasis, common symptoms include dyspnea, cough, sputum production, fatigue, and reduced activity and social participation 4 . While CF and non‐CF bronchiectasis cause similar symptoms like chronic cough, sputum production, chest pain, and decrease in exercise tolerance, non‐CF bronchiectasis is an underestimated chronic disorder and there is limited evidence about the morbidity in this patient population 5…”

Deep learning to automate Brasfield chest radiographic scoring for cystic fibrosis

“…Unlike other Brasfield features, "large lesions" comprise a relatively heterogeneous group of potential abnormalities. Furthermore, while other Brasfield features (e.g., linear markings and nodular cystic lesions) tend to increase in severity concurrently with progressive CF lung disease, "large lesions" are more sporadic and asyn- 0.08 −1.21 p = 0.16 p = 0.83 p < 0.001 † p = 0.006 † p = 0.89 p = 0.01 † Model v. Rad 1,3-5 Avg 0.26 0.16 0.00 −0.20 0.02 −0.24 Rad 2 v. Rad 1,3-5 Avg 0.11 0.06 0.52 −0.03 0.10 −0.74 p = 0.006 † p = 0.039 † p < 0.001 † p = 0.18 p = 0.84 p = 0.28 Model v. Rad 1-2,4-5 Avg 0.26 0.14 −0.12 −0.20 −0.01 −0.07 Rad 3 v. Rad 1-2,4-5 Avg 0.11 −0.06 −0.07 −0.04 −0.05 0.12 p = 0.003 † p = 0.002 † p = 0.27 p < 0.001 † p = 0.11 p = 0.03 † Model v. Rad chronous, potentially reflect more acute disease that could improve or even disappear with treatment [31][32][33]. Moreover, as opposed to other Brasfield categories in which severity increases are gradual and separated by only 1 point, large lesions can only be scored 0, 3, or 5; thus, any "errors" in large lesion classification appear more pronounced in assessing the model's overall performance.…”

Effectiveness of Hypertonic Saline Nebulization in Airway Clearance in Children with Non-Cystic Fibrosis Bronchiectasis: A randomized control trial

“…7 A complex interplay between infection and inflammation creates a pro-inflammatory vicious cycle that progressively destructs pulmonary architecture leading to chronic bronchial dilatation which is the hallmark of bronchiectasis. 8 With this pathophysiological background, management is directed at breaking this vicious cycle and facilitating the muco-ciliary clearance of secretions which will hinder the on-going bronchial damage. .…”