Rationale: Testing for underlying etiology is a key part of bronchiectasis management, but it is unclear whether the same extent of testing is required across the spectrum of disease severity. Objectives: The aim of the present study was to identify the etiology of bronchiectasis across European cohorts and according to different levels of disease severity. Methods: We conducted an analysis of seven databases of adult outpatients with bronchiectasis prospectively enrolled at the bronchiectasis clinics of university teaching hospitals in Monza, Italy; Dundee and Newcastle, United Kingdom; Leuven, Belgium; Barcelona, Spain; Athens, Greece; and Galway, Ireland. All the patients at every site underwent the same comprehensive diagnostic workup as suggested by the British Thoracic Society. Measurements and Main Results: Among the 1,258 patients enrolled, an etiology of bronchiectasis was determined in 60%, including postinfective (20%), chronic obstructive pulmonary disease related (15%), connective tissue disease related (10%), immunodeficiency related (5.8%), and asthma related (3.3%). An etiology leading to a change in patient’s management was identified in 13% of the cases. No significant differences in the etiology of bronchiectasis were present across different levels of disease severity, with the exception of a higher prevalence of chronic obstructive pulmonary disease–related bronchiectasis (P < 0.001) and a lower prevalence of idiopathic bronchiectasis (P = 0.029) in patients with severe disease. Conclusions: Physicians should not be guided by disease severity in suspecting specific etiologies in patients with bronchiectasis, although idiopathic bronchiectasis appears to be less common in patients with the most severe disease.

The goal of this study was to develop a simplified radiological score that could assess clinical disease severity in bronchiectasis. The Bronchiectasis Radiologically Indexed CT Score (BRICS) was devised based on a multivariable analysis of the Bhalla score and its ability in predicting clinical parameters of severity. The score was then externally validated in six centers in 302 patients. A total of 184 high-resolution CT scans were scored for the validation cohort. In a multiple logistic regression model, disease severity markers significantly associated with the Bhalla score were percent predicted FEV₁, sputum purulence, and exacerbations requiring hospital admission. Components of the Bhalla score that were significantly associated with the disease severity markers were bronchial dilatation and number of bronchopulmonary segments with emphysema. The BRICS was developed with these two parameters. The receiver operating-characteristic curve values for BRICS in the derivation cohort were 0.79 for percent predicted FEV₁, 0.71 for sputum purulence, and 0.75 for hospital admissions per year; these values were 0.81, 0.70, and 0.70, respectively, in the validation cohort. Sputum free neutrophil elastase activity was significantly elevated in the group with emphysema on CT imaging. A simplified CT scoring system can be used as an adjunct to clinical parameters to predict disease severity in patients with idiopathic and postinfective bronchiectasis.

Bronchiectasis (BE) is a chronic structural lung disease with frequent exacerbations, some of which require hospital admission though no clear associated factors have been identified. We aimed to evaluate factors associated with hospitalization due to exacerbations during a 1-year follow-up period. A prospective observational study was performed in patients recruited from specialized BE clinics. We considered all exacerbations diagnosed and treated with antibiotics during a follow-up period of 1 year. The protocol recorded baseline variables, usual treatments, Bronchiectasis Severity Index (BSI) and FACED scores, comorbid conditions and prior hospitalizations. Two hundred and 65 patients were recruited, of whom 162 required hospital admission during the follow-up period. Independent risk factors for hospital admission were age, previous hospitalization due to BE, use of proton pump inhibitors, heart failure, FACED and BSI, whereas pneumococcal vaccination was a protective factor. The area under the receiver operator characteristic curve (AUC) was 0.799 for BSI model was 0.799, and 0.813 for FACED model. Previous hospitalization, use of proton pump inhibitors, heart failure along with BSI or FACED scores is associated factors for developing exacerbations that require hospitalization. Pneumococcal vaccination was protective. This information may be useful for the design of preventive strategies and more intensive follow-up plans.

Bronchiectasis (BE) is a chronic and heterogeneous respiratory disease that requires a multidimensional scoring system to properly assess severity. The aim of this study was to compare the severity stratification by 2 validated scores (BSI and FACED) in a BE cohort and to determine their predictive capacity for exacerbations and hospitalizations. Moreover, we proposed a modified version of FACED which was created to better predict the risk of exacerbations in clinical practice. We performed a prospective cohort study including BE patients >18 years old with a follow-up period of 1-year. One-hundred eighty-two patients (40% males; mean age 68) were studied. Patients were stratified according to the number of exacerbations during the follow-up, and according to BSI and FACED scores. BSI classified most of our patients as severe 99 (54.4%) or moderate 47 (25.8%), while FACED mainly classified as mild 108 (59.3%) or moderate 61 (33.5%). BSI and FACED showed an area under ROC curve (AUC) for exacerbations of 0.808 and 0.734; and for hospitalizations (due to BE exacerbations) of 0.893 and 0.809, respectively. Subsequently, we modified FACED by adding previous exacerbations (Exa-FACED) and this new score classified patients as mild 48.4%, moderate 34.6% and severe 17.0%, with an improved AUC for exacerbations (0.760) and hospitalizations (0.820). Despite previous validations of BSI and FACED, they classified our patients very differently. As expected, FACED showed poor prognostic capacity for exacerbations. We support the Exa-FACED score to predict the risk future exacerbations for been easy to use in clinical practice.