Bronchiectasis Severity Research Articles

The Bronchiectasis Severity Index and FACED score for assessment of the severity of bronchiectasis

IntroductionBronchiectasis (BC) is a multidimensional and etiologically diverse disease and, therefore, no single parameter can be used to determine its overall severity and prognosis. In this regard, two different validated scores are currently used to assess the severity of non-cystic fibrosis bronchiectasis (NCFB): the FACED score and the Bronchiectasis Severity Index (BSI). ObjectiveTo describe the etiology of NCFB and compare the results of the assessment of NCFB severity obtained via FACED and BSI scores. MethodsRetrospective study of demographic and clinical data of a convenience sample of NCFB patients attending the Functional Breathing Re-adaptation appointment at the Pneumology B Unit, University Hospital Center of Coimbra. All patients underwent evaluation of the variables incorporated in the FACED score (FEV1% predicted, age, chronic colonization by Pseudomonas aeruginosa, radiological extent of the disease, and dyspnea) and in the BSI (age, body mass index, FEV1% predicted, hospitalization and exacerbations before study, dyspnea, chronic colonization by P. aeruginosa and other microrganisms, and radiological extent of the disease). Statistical analysis of the data was performed using Microsoft Excel® and IBM SPSS® v23. ResultsThe sample included 40 patients, 22 females and 18 males, aged 39–87 years. Regarding the etiology of NCFB, we found: idiopathic (60%), post-infectious (20%), sequelae of pulmonary tuberculosis (12.5%) and primary immunodeficiency related (7.5%).According to the FACED score we found 20 patients (50%) with mild BC, 15 patients (37.5%) with moderate and 5 patients (12.5%) with severe BC. The frequency of patients with low, intermediate and high BSI was 13 (32.5%), 13 (32.5%) and 14 (35%), respectively in relation to derived BSI, Moreover, we observed a weak but statistically significant association between FACED and BSI scores: Fisher's exact test (p=0.004), tau-b de Kendall (0.469; p=0.001). The Kappa test (0.330; p=0.002) also shows us that there is 55% agreement between the two scales. ConclusionThere is a small but significant correlation between the two scales: a tendency is observed for patients to be classified with a higher BSI compared to the FACED score. This can be explained by the fact that BSI (and not FACED) evaluates parameters including BMI, hospitalization and exacerbations before study, chronic colonization by other microorganisms and development of cystic bronchiectasis. Further studies should address how these scores may impact clinical practice.

COPD-bronchiectasis overlap syndrome: Are there clinical, physiological and radiological synergistic effects?

Introduction: The coexistence of chronic obstructive pulmonary disease (COPD) and bronchiectasis is increasingly diagnosed in clinical practice; however this overlap is not well studied. Aim: To study the impact of overlap between of COPD and bronchiectasis on patient clinical, physiological and radiological indices as well as health related quality of life (HRQOL). Patients and methods: One hundred eighty-four patients were included; 68 patients had COPD, 68 had bronchiectasis, and 48 had overlapping COPD and bronchiectasis. Clinical assessment, arterial blood gas (ABG) analysis, pulmonary function tests, and high resolution computed tomography (HRCT) chest with Bhalla scoring were done. HRQOL were assessed using the arabic version of St. George Respiratory Questionnaire (SGRQ). Results: Dyspnea scale significantly increased in COPD, and overlapping COPD and bronchiectasis groups compared to bronchiectasis group. There was no significant difference between the three groups regarding ABG values, spirometeric parameters, diffusing capacity for carbon monoxide (DLCO) and SGRQ score. Bronchiectasis group had a significantly higher total Bhalla score with significant deterioration of spirometeric parameters and ABG values with increasing radiological extent and severity of bronchiectasis. Conclusion: The overlap between COPD and bronchiectasis did not significantly impair physiological parameters or HRQOL in comparison to either disease alone.

Clinical Determinants of Incremental Shuttle Walk Test in Adults with Bronchiectasis.

Exercise capacity is impaired in patients with bronchiectasis. Incremental shuttle walk test (ISWT) stresses cardiorespiratory system physiologically to symptom-limited maximal exercise capacity. The purpose of this study was to investigate the clinical determinants of ISWT in adults with non-cystic fibrosis (CF) bronchiectasis. Forty-one clinically stable bronchiectasis patients aged 18-72years (27 females, 14 males) participated in the study. Subjects' demographics and physical characteristics were recorded. Bronchiectasis Severity Index was used to identify disease severity. Pulmonary function test was performed. Dyspnea perception was assessed using the modified Medical Research Council Dyspnea Scale. Maximum inspiratory and expiratory pressures were measured. Peripheral muscle strength using a hand held dynamometer was measured. ISWT was performed to determine exercise capacity. Fatigue Severity Scale, Hospital Anxiety and Depression Scale, Leicester Cough Questionnaire were used to determine fatigue, psychosocial status, and quality of life. Patients' mean ISWT distance was 469.5m. The ISWT distance was significantly related with age (r = - 0.472), height (r = 0.469), gender (r = 0.520), FEV1 (r = 0.651), and FVC (r = 0.545, p < 0.05). Quadriceps muscle strength was higher in males (p = 0.001) as compared to females. Age and gender were identified as independent predictors of the ISWT, explaining 42% of variance in ISWT distance (r = 0.649, r2 = 0.421, F(2,38) = 13.794, p < 0.001). The clinical determinants of ISWT in clinically stable patients with non-CF bronchiectasis are age and gender. Pulmonary function, dyspnea perception, muscle strength, disease severity, fatigue, psychosocial factors, and health-related quality of life seems to have an independent effect on ISWT in this group of patients with bronchiectasis.

Co-existence of COPD and bronchiectasis: a risk factor for a high ratio of main pulmonary artery to aorta diameter (PA:A) from computed tomography in COPD patients.

BackgroundPulmonary vascular disease, especially pulmonary hypertension, is an important complication of COPD. Bronchiectasis is considered not only a comorbidity of COPD, but also a risk factor for vascular diseases. The main pulmonary artery to aorta diameter ratio (PA:A ratio) has been found to be a reliable indicator of pulmonary vascular disease. It is hypothesized that the co-existence of COPD and bronchiectasis may be associated with relative pulmonary artery enlargement (PA:A ratio >1).MethodsThis retrospective study enrolled COPD patients from 2012 through 2016. Demographic and clinical data were collected. Bhalla score was used to determine the severity of bronchiectasis. Patient characteristics were analyzed in two ways: the high (PA:A >1) and low (PA:A ≤1) ratio groups; and COPD with and without bronchiectasis groups. Logistic regression analysis was used to assess risk factors for high PA:A ratios.ResultsIn this study, 480 COPD patients were included, of whom 168 had radiographic bronchiectasis. Patients with pulmonary artery enlargement presented with poorer nutrition (albumin, 35.6±5.1 vs 38.3±4.9, P<0.001), lower oxygen partial pressure (74.4±34.5 vs 81.3±25.4, P<0.001), more severe airflow obstruction (FEV1.0, 0.9±0.5 vs 1.1±0.6, P=0.004), and a higher frequency of bronchiectasis (60% vs 28.8%, P<0.001) than patients in the low ratio group. Patients with both COPD and bronchiectasis had higher levels of systemic inflammation (erythrocyte sedimentation rate, P<0.001 and fibrinogen, P=0.006) and PA:A ratios (P<0.001). A higher PA:A ratio was significantly closely correlated with a higher Bhalla score (r=0.412, P<0.001). Patients with both COPD and bronchiectasis with high ratios presented higher levels of NT-proBNP (P<0.001) and systolic pulmonary artery pressure (P<0.001). Multiple logistic analyses have indicated that bronchiectasis is an independent risk factor for high PA:A ratios in COPD patients (OR =3.707; 95% CI =1.888–7.278; P<0.001).ConclusionBronchiectasis in COPD has been demonstrated to be independently associated with relative pulmonary artery enlargement.

The BRICS (Bronchiectasis Radiologically Indexed CT Score): A Multicenter Study Score for Use in Idiopathic and Postinfective Bronchiectasis

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.

Subclinical atherosclerosis in adults with steady-state bronchiectasis: A case-control study.

Bronchiectasis has been associated with increased risks of cardiovascular disease, in which early-stage endothelial dysfunction might have played pivotal roles. We aimed to investigate endothelial function in bronchiectasis patients, by measuring flow-mediated dilatation (FMD) and carotid intima-media thickness (CIMT) as compared with control subjects, and to elucidate the impact of different risk factors on subclinical atherosclerosis. The study included 80 bronchiectasis patients and 80 age- and sex-matched healthy subjects. Vascular endothelial function was evaluated with FMD in the brachial artery in response to reactive hyperemia, and CIMT was measured with high-resolution ultrasonography. Disease severity was evaluated with Bronchiectasis Severity Index and FACED scores. Demographic, disease duration, radiology, spirometry, sputum bacteriology and systemic inflammatory indices were also assessed. FMD was significantly lower in bronchiectasis patients than in control subjects (8.92±2.70% vs. 11.17±3.44%, P<0.001). FMD significantly correlated with Bronchiectasis Severity Index (rho=-0.60, P<0.001) and FACED score (rho=-0.39, P<0.001). In multivariate regression analysis, age, body-mass index, Pseudomonas aeruginosa colonization and high-resolution computed tomography scores were independent factors influencing on the FMD in bronchiectasis patients, even after adjustment for other clinical variables. No significant difference in CIMT was detected between bronchiectasis patients and healthy subjects (P>0.05). Compared with healthy subjects, bronchiectasis patients have greater risks of endothelial dysfunction which is independent of previously well-studied risk factors, calling for the vigilance to screen early for vascular changes to minimize the future risks of cardiovascular events.

Impact of chronic Pseudomonas aeruginosa infection on health-related quality of life in Mycobacterium avium complex lung disease

BackgroundIn bronchiectasis patients, chronic Pseudomonas aeruginosa (PA) infection has been associated with worse health-related quality of life (HRQL), but little is known about Mycobacterium avium complex lung disease (MACLD) patients in this context. This study aimed to evaluate HRQL and investigate the impact of chronic PA infection in MACLD patients.MethodsThis cross-sectional study was conducted using the Registry of Prospective Cohort Study including MACLD patients. The 36-item Short-Form health survey (SF-36) and St. George’s Respiratory Questionnaire (SGRQ) were administered to assess clinical outcomes. Clinical variables included treatment and sputum culture status, pulmonary function tests, cavitary lesions, and modified Reiff scores on high-resolution computed tomography.ResultsThe study included 244 MACLD patients (median age, 68 years; 196 women), 19 of whom had chronic PA infection. Modified Reiff score was higher in patients with chronic infection than in those without (P = 0.028). Regarding SF-36 scores, physical functioning subscale scores were significantly lower in patients with chronic infection (P = 0.029). Additionally, SGRQ symptoms, impact, and total scores were significantly higher in patients with chronic infection. During analysis of covariance comparisons, SGRQ symptoms and impact scores were significantly higher for patients with chronic infection (P = 0.043 and 0.021, respectively).ConclusionsMACLD patients with chronic PA infection exhibited significantly higher SGRQ scores, indicating impaired HRQL. Chronic PA infection was significantly associated with the severity of bronchiectasis.

Correlates and assessment of excess cardiovascular risk in bronchiectasis.

Patients with bronchiectasis are at increased risk of cardiovascular disease. We aimed to identify factors associated with elevated cardiovascular risk in bronchiectasis, measured using aortic stiffness and cardiac biomarkers. In addition, we sought to compare these direct measures against calculated QRISK2 scores.Aortic stiffness, cardiac biomarkers and systemic inflammation were measured in 101 adults with stable bronchiectasis. In addition, clinical and demographic data were collected to allow calculation of QRISK2score and the bronchiectasis severity index (BSI) for each patient.The BSI score correlated with measured cardiovascular risk assessments, partly due to greater exacerbation frequency and lower forced expiratory volume in 1 s. Pulse-wave velocity was significantly higher in frequent exacerbators (≥3 events·year-1) than infrequent exacerbators (<3 events·year-1; 10.5 versus 9.2 m·s-1, p=0.01). In addition, frequent exacerbators had elevated serum C-reactive protein concentration, suggesting increased systemic inflammation (4.8 versus 2.2 mg·L-1, p=0.005). QRISK2 systematically underestimated cardiovascular risk in this population (median change in relative risk 1.29). Underestimation was associated with frequent exacerbations and male sex.Patients with bronchiectasis have greater cardiovascular risk than published reference populations. Excess cardiovascular risk is associated with exacerbation frequency and impaired lung function. Cardiovascular risk assessment in bronchiectasis should be individualised, as calculation tools are likely to underestimate the risk in this population.

Burkholderia cepacia complex in cystic fibrosis in a Brazilian reference center.

The Burkholderia cepacia complex (BCC) can cause a severe decline in lung function in cystic fibrosis (CF). Our objective was to determine the BCC prevalence and to evaluate its clinical impact on CF. Clinical and laboratory variables were determined for CF patients with BCC (Group-A=50 patients) and without BCC (Group-B=134 patients). The microorganisms were identified by biochemical tests, the Vitek2®Compact test, recA-PCR and recA-nested-PCR with species-specific primers and DNA sequencing. The patients were evaluated by the Shwachman-Kulczycki score (SKCS), Bhalla score (BS), spirometry and body mass index (BMI). The BCC prevalence was 22.5%. The most common species were Burkholderia multivorans (30%), Burkholderia cepacia (24%), Burkholderia cenocepacia IIIA (10%), B. cenocepacia IIIB (2%) and Burkholderia vietnamiensis (2%). There was difference between the groups in nutritional status (p=0.02) and general activity (p=0.026). There was difference in total BS points (p=0.04) and the following parameters: bronchiectasis severity (p=0.007), peribronchial thickening (p=0.013), bronchiectasis extent (p=0.01) and general aspects of the affected bronchial zone (p=0.02). The respiratory disorder classifications were as follows: obstructive-4.8% (Group-A) and 23.8% (Group-B); restrictive-9.5% (Group-A and Group-B); obstructive+restrictive-19% (Group-A) and 1.6% (Group-B); and obstructive+restrictive with a decreased forced expiratory flow-47.6% (Group-A) and 30.2% (Group-B) (p=0.02). Nutritional status was a minor contributing factor to weight, height and BMI in the Group-A (p=0.02). The BCC prevalence, particularly the prevalence of B. multivorans, was higher in this study. The SKCS, BS, spirometry and nutritional status results showed that BCC has a negative impact on clinical status. Phenotypic methods are useful for the identification of presumptive BCC. The Vitek2®Compact test showed accuracy in BCC identification. PCR, nested-PCR, and recA sequencing showed specificity in BCC species identification.

Factors associated with hospitalization in bronchiectasis exacerbations: a one-year follow-up study

BackgroundBronchiectasis (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.MethodsA 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.ResultsTwo 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.ConclusionsPrevious 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.

Anxiety and depression in adult outpatients with bronchiectasis: Associations with disease severity and health-related quality of life.

Anxiety and depression might frequently affect bronchiectasis patients, but data in Chinese patients, including their association with disease severity assessed with Bronchiectasis Severity Index (BSI) and FACED score, are limited. To investigate the rate, risk factors, association with disease severity and impact of anxiety and depression on health-related quality of life (HRQoL) in adult outpatients with steady-state bronchiectasis. This cross-sectional study included 163 outpatients (102 females; mean age, 45.8 years) and 80 healthy subjects (47 females; mean age, 47.1 years). Demographic, clinical indices, radiology, spirometry, aetiology, sputum bacteriology, Hospital Anxiety and Depression Scales (HADS), Pittsburgh Sleep Quality Index (PSQI) and St. George's Respiratory Questionnaire (SGRQ) were assessed. Patients with steady-state bronchiectasis had a higher rate of depression (HADS-depression >7) (30.1% vs 10.0%, P = .001) and anxiety (HADS-anxiety >7; 39.9% vs 6.3%, P < .001) compared with healthy subjects. Notably, no significant differences in the rate of anxiety and depression were found across different disease severity, assessed with BSI and FACED score (all P > .05). In multivariate model, factors associated with anxiety included younger age (OR = 1.05), education below college graduate (OR = 4.55) and sleep disturbance (PSQI ≥ 6; OR = 2.95); whereas sleep disturbance was the sole factor associated with depression (OR = 5.98). Patients with either depression or anxiety had more markedly impaired HRQoL affecting most domains than those without. Anxiety and depression are common in bronchiectasis and can negatively affect HRQoL, but not related to disease severity. Prompt assessment and treatment of these mental disorders, regardless of bronchiectasis severity, are advocated and might improve HRQoL.

Human Immunodeficiency Virus-Associated Chronic Lung Disease in Children and Adolescents in Zimbabwe: Chest Radiographic and High-Resolution Computed Tomographic Findings.

Chronic respiratory symptoms are common among children living with human immunodeficiency virus (HIV). We investigated the radiological features of chronic lung disease in children aged 6-16 years receiving antiretroviral therapy for ≥6 months in Harare, Zimbabwe. Consecutive participants from a HIV clinic underwent clinical assessment and chest radiography. Participants with an abnormal chest radiograph (assessed by a clinician) and/or those meeting a clinical case definition for chronic lung disease underwent high-resolution computed tomography (HRCT). Radiological studies were scored independently and blindly by 2 thoracic radiologists. Relationships between radiological abnormalities and lung function were examined. Among 193 participants (46% female; median age, 11.2 years; interquartile range, 9.0-12.8 years), the median CD4 cell count was 720/µL (473-947/µL), and 79% had a human immunodeficiency virus (HIV) load of <400 copies/mL. The most common chest radiographic finding was ring/tramline opacities (55 of 193 participants; 29%). HRCT scans were evaluated in 84 participants (69%); decreased attenuation (present in 43%) was the dominant abnormality seen. The extent of decreased attenuation was strongly correlated with both the severity and extent of bronchiectasis (rs = 0.68 and P < .001 for both). The extent of decreased attenuation was also negatively correlated with forced expiratory volume in first second of expiration (rs = -0.52), forced vital capacity (rs = -0.42), and forced expiratory flow, midexpiratory phase (rs = -0.42) (P < .001 for all). The HRCT findings strongly suggest that obliterative bronchiolitis may be the major cause of chronic lung disease in our cohort. Further studies to understand the pathogenesis and natural history are urgently needed.

Unclassifiable-interstitial lung disease: Outcome prediction using CT and functional indices.

Unclassifiable-interstitial lung disease (uILD) represents a heterogeneous collection of pathologies encompassing those fibrosing lung diseases which do not fulfill current diagnostic criteria. We evaluated baseline and longitudinal functional and CT (visual and quantitative computer [CALIPER] analysis) variables to identify outcome predictors in uILD. Consecutive patients with uILD on multidisciplinary review (n=95) had baseline functional (FVC, DLco, CPI [composite physiologic index]) and CT features (visual evaluation: CT pattern, fibrosis extent, honeycombing presence, traction bronchiectasis severity, pulmonary artery (PA) diameter; CALIPER evaluation: fibrosis extent, pulmonary vessel volume (PVV)) examined in univariate and multivariate Cox regression models. Change in functional and CT variables were examined in a patient subset (n=37), to identify indicators of outcome. On univariate analysis, CPI was the most powerful functional predictor of mortality (p<0.0001). Visual traction bronchiectasis (p<0.0001), PA diameter (p<0.0001) and honeycombing presence (p=0.0001) and CALIPER PVV (p=0.0003) were the strongest CT outcome predictors. On multivariate analysis of baseline indices, traction bronchiectasis (p=0.003), PA diameter (p=0.003) and CPI (p=0.0001) independently predicted mortality. Colinearity with functional indices precluded the evaluation of CALIPER PVV in multivariate models. On evaluation of longitudinal variables, increasing CALIPER fibrosis extent was the strongest outcome predictor, and remained so following adjustment for baseline disease severity, and when FVC declines were marginal. In uILD patients, CPI, traction bronchiectasis severity and PA diameter independently predicted outcome at baseline. Increasing fibrosis extent measured by CALIPER was the most powerful index of outcome regardless of baseline disease severity and strongly predicted outcome in patients with marginal FVC declines.

Investigation of bronchiectasis in severe uncontrolled asthma.

The presence of bronchiectasis in patients with asthma varies in different reports, while a clear aetiological relation has not been precisely established. To investigate the presence of bronchiectasis in patients with severe uncontrolled asthma and examine whether they contribute to the severity of asthma. Patients with severe asthma were prospectively recruited. HRCT of the chest was performed to identify and grade bronchiectasis using the 'Smith' radiology scale. Investigation of the underlying cause was carried out for patients with bronchiectasis in order to exclude aetiologies other than asthma. The Statistical Package for the Social Sciences (SPSS), version 21, was used. Forty patients were studied, 28 women, mean age (±SD) 57.9 years (±12.4). Mean ACT score was 14.2(±4.9). Main symptoms were: wheezing (95%), cough (92%), dysponea (92%) and sputum production (72%). Mean duration of asthma was 16.5(±11.5) years, exacerbations: 4.4(±2.7)/year. In 27 patients (67.5%) bronchiectasis was diagnosed. In nine patients (22.5%) pathogens were cultured in sputum (mainly Pseudomonas aeruginosa, Haemophilus influenzae). Patients with sputum production and pathogens in sputum cultures had a higher Smith score compared to those without expectoration and without pathogens, respectively (P = .005, P < .0001). No correlation was found between the extent of bronchiectasis and lung function. The radiological severity of bronchiectasis was correlated with the antibiotic courses/year (P = .002). Bronchiectasis is common in patients with severe asthma. Sputum production and pathogen isolation in sputum may indicate the presence of bronchiectasis which seems to contribute to the severity of asthma.

Diagnosis of bronchiectasis and airway wall thickening in children with cystic fibrosis: Objective airway-artery quantification

ObjectivesTo quantify airway and artery (AA)-dimensions in cystic fibrosis (CF) and control patients for objective CT diagnosis of bronchiectasis and airway wall thickness (AWT).MethodsSpirometer-guided inspiratory and expiratory CTs of 11 CF and 12 control patients were collected retrospectively. Airway pathways were annotated semi-automatically to reconstruct three-dimensional bronchial trees. All visible AA-pairs were measured perpendicular to the airway axis. Inner, outer and AWT (outer−inner) diameter were divided by the adjacent artery diameter to compute AinA-, AoutA- and AWTA-ratios. AA-ratios were predicted using mixed-effects models including disease status, lung volume, gender, height and age as covariates.ResultsDemographics did not differ significantly between cohorts. Mean AA-pairs CF: 299 inspiratory; 82 expiratory. Controls: 131 inspiratory; 58 expiratory. All ratios were significantly larger in inspiratory compared to expiratory CTs for both groups (p<0.001). AoutA- and AWTA-ratios were larger in CF than in controls, independent of lung volume (p<0.01). Difference of AoutA- and AWTA-ratios between patients with CF and controls increased significantly for every following airway generation (p<0.001).ConclusionDiagnosis of bronchiectasis is highly dependent on lung volume and more reliably diagnosed using outer airway diameter. Difference in bronchiectasis and AWT severity between the two cohorts increased with each airway generation.Key points• More peripheral airways are visible in CF patients compared to controls.• Structural lung changes in CF patients are greater with each airway generation.• Number of airways visualized on CT could quantify CF lung disease.• For objective airway disease quantification on CT, lung volume standardization is required.

Anti-Pseudomonas aeruginosa IgG antibodies and chronic airway infection in bronchiectasis

BackgroundIdentification of chronic Pseudomonas aeruginosa (PA) infection is important in the management of bronchiectasis, but requires repeated sputum sampling. We hypothesized that serum anti-PA IgG antibodies could diagnose chronic PA infection at a single visit. MethodsClinically stable bronchiectasis patients were studied prospectively. Chronic PA infection was defined as 2 or more positive sputum samples at least 3 months apart and/or failure to clear PA following eradication treatment. Baseline serum anti-PA IgG was determined by a validated ELISA kit. ResultsA total of 408 patients were included. Sixty of them (14.7%) had chronic PA infection and had higher anti-PA IgG levels (median 6.2 vs. 1.3 units, p < 0.001). Antibody levels showed direct significant correlations with exacerbation frequency, the bronchiectasis severity index and sputum inflammatory markers. Fifty-seven patients with chronic PA infection had a positive test, giving 95% sensitivity, 74.4% specificity and AUROC of 0.87. During follow-up, 38 patients had a new PA isolation. Eradication at 12 months was achieved in 89.5% of subjects with a negative antibody test and 15.8% of patients with a positive test. ConclusionsAnti-PA IgG test is highly accurate to detect chronic PA infection in bronchiectasis patients. In addition, it may be a marker of disease severity and treatment response.

Patterns of Disease in Patients with Middle-Lobe Predominant Bronchiectasis

Background: Middle-lobe predominant bronchiectasis affecting the right middle-lobe and/or lingula (RMLP) is classically described in asthenic, elderly females with skeletal abnormalities or associated nontuberculous mycobacterial (NTM) infection. Objectives: We aimed to evaluate the frequency and clinical characteristics of patients with an RMLP phenotype in a cohort of newly diagnosed bronchiectasis patients and determine associations with disease severity. Methods: A retrospective observational cross-sectional cohort study of consecutive bronchiectasis patients in our institution was performed. Data were collected on baseline variables, microbiology status, lung function, and radiology according to the modified Bhalla score. Disease severity was assessed using bronchiectasis severity index (BSI) and FACED severity scores. Results: Of 81 patients (mean age [SD] 62.6 [12.4], females 55 [67.9%], BMI 26.9 [5.7%]), 20 (24.7%) had RMLP disease. These patients were significantly younger, female, and with lower BMIs than patients with the classical bronchiectasis phenotype (p = 0.03, 0.01, and p <0.01, respectively). Fewer symptoms of cough and daily sputum (p = 0.01 and <0.01), prior exacerbation frequency (p = 0.03), and higher baseline forced expiratory volume (p = 0.04) were noted. A higher incidence of NTM at diagnosis was demonstrated (p = 0.01). BSI and FACED severity scores in RMLP patients were significantly lower than their counterparts (both p < 0.001). Conclusions: The RMLP phenotype is associated with younger patients than classically described in the literature. An increased rate of NTM infection in this phenotype was noted, particularly in females, but much lower than previously described. Lung function and disease severity scores in this patient group are relatively normal, suggesting a milder phenotype in patients with this form of the disease.

The heterogeneity of systemic inflammation in bronchiectasis

BackgroundSystemic inflammation in bronchiectasis is poorly studied in relation to aetiology and severity. We hypothesized that molecular patterns of inflammation may define particular aetiology and severity groups in bronchiectasis. MethodWe assayed blood concentrations of 31 proteins from 90 bronchiectasis patients (derivation cohort) and conducted PCA to examine relationships between these markers, disease aetiology and severity. Key results were validated in two separate cohorts of 97 and 79 patients from other centres. ResultsThere was significant heterogeneity in protein concentrations across the derivation population. Increasing severity of bronchiectasis (BSI) was associated with increasing fibrinogen (rho = 0.34, p = 0.001 –validated in a second cohort), and higher fibrinogen was associated with worse lung function, Pseudomonas colonisation and impaired health-status. There were generally similar patterns of inflammation in patients with idiopathic and post-infectious disease. However, patients with primary immunodeficiency had exaggerated IL-17 responses, validated in a second cohort (n = 79, immunodeficient 12.82 pg/ml versus idiopathic/post-infectious 4.95 pg/ml, p = 0.001), and thus IL-17 discriminated primary immunodeficiency from other aetiologies (AUC 0.769 (95%CI 0.661–0.877)). ConclusionBronchiectasis is associated with heterogeneity of systemic inflammatory proteins not adequately explained by differences in disease aetiology or severity. More severe disease is associated with enhanced acute-phase responses. Plasma fibrinogen was associated with bronchiectasis severity in two cohorts, Pseudomonas colonisation and health status, and offers potential as a useful biomarker.

Bronchiectasis: Phenotyping a Complex Disease

ABSTRACTBronchiectasis is a long-neglected disease currently experiencing a surge in interest. It is a highly complex condition with numerous aetiologies, co-morbidities and a heterogeneous disease presentation and clinical course. The past few years have seen major advances in our understanding of the disease, primarily through large real-life cohort studies. The main outcomes of interest in bronchiectasis are symptoms, exacerbations, treatment response, disease progression and death. We are now more able to identify clearly the radiological, clinical, microbiological and inflammatory contributors to these outcomes. Over the past couple of years, multidimensional scoring systems such as the Bronchiectasis Severity Index have been introduced to predict disease severity and mortality. Although there are currently no licensed therapies for bronchiectasis, an increasing number of clinical trials are planned or ongoing. While this emerging evidence is awaited, bronchiectasis guidelines will continue to be informed largely by real-life evidence from observational studies and patient registries. Key developments in the bronchiectasis field include the establishment of international disease registries and characterisation of disease phenotypes using cluster analysis and biological data.

Chronic rhinosinusitis is associated with higher prevalence and severity of bronchiectasis in patients with COPD.

Background and purposeBronchiectasis revealed by high-resolution computed tomography (HRCT) is common in chronic obstructive pulmonary disease (COPD), but the causes and risk factors remain to be determined. Chronic rhinosinusitis (CRS) is closely associated with bronchiectasis or COPD, but whether it is associated with comorbid bronchiectasis in COPD (COPD-Bx) is unknown.Patients and methodsPatients with stable COPD were enrolled consecutively and evaluated for the presence of CRS by questionnaire and paranasal sinus computed tomography. The presence and severity of bronchiectasis on lung HRCT were evaluated by the Smith and severity scores. COPD symptoms were evaluated by COPD Assessment Test (CAT) and Modified British Medical Research Council Questionnaire. The sputum cell differentials and concentrations of interleukin (IL)-6, IL-8, IL-5, matrix metalloproteinases-9 (MMP-9), and tissue inhibitor of matrix metalloproteinases-1 were measured.ResultsWe enrolled 136 patients with stable COPD, of which 66 (48.5%) were diagnosed with CRS according to the European Position Paper on Rhinosinusitis and Nasal Polyps (EP3OS) criteria. The prevalence of bronchiectasis was 57.6% in patients with CRS, but 37.1% in those without CRS (P=0.017). COPD-Bx patients with CRS showed a significantly higher severity score of bronchiectasis than those without CRS (P=0.034). COPD patients with CRS had a higher percentage of eosinophils, higher levels of IL-8, IL-6, and MMP-9 in sputum as compared to those without CRS. In COPD-Bx patients with CRS, the percentage of eosinophils and the levels of IL-6 and MMP-9 in sputum were increased as compared to those without CRS. In all the subjects, Sino-Nasal Outcome Test-20 correlated with CAT score (r=0.315, P<0.01) and in COPD patients with CRS, Lund–MacKay scores correlated with forced expiratory volume in 1 s (% pred) (r=−0.251, P<0.05).ConclusionsCRS was associated with COPD-Bx and this was probably due to increased airway inflammation.