Bronchiectasis Severity Index Score Research Articles

Brensocatib in non–cystic fibrosis bronchiectasis: ASPEN protocol/baseline characteristics

Brensocatib is an investigational, oral, reversible inhibitor of dipeptidyl peptidase-1 shown to prolong time to first exacerbation in adults with bronchiectasis. Outlined here are the clinical trial design, and baseline characteristics and treatment patterns of adult patients enrolled in the phase 3 ASPEN trial (NCT04594369).A global study enrolling patients with a clinical history consistent with bronchiectasis (cough, chronic sputum production, and/or recurrent respiratory infections), diagnosis confirmed radiologically, and ≥2 exacerbations in the prior 12 months, ASPEN was designed to evaluate the impact of two brensocatib doses (10 mg and 25 mg) on exacerbation rate over a 52-week treatment periodversusplacebo. Comprehensive clinical data, including demographics, disease severity, lung function,Pseudomonas(P.) aeruginosastatus, and quality of life, were collected at baseline.1682 adults from 35 countries were randomised from December 2020 to March 2023. Mean age was 61.3 years and 64.7% were female. Approximately 70% had moderate-to-severe Bronchiectasis Severity Index (BSI) scores, 29.3% had ≥3 exacerbations in the prior 12 months, and 35.7% were positive forP. aeruginosa. Mean BSI scores were highest in Australia/New Zealand (8.3) and lowest in Latin America (5.9). Overall, the most common aetiology was idiopathic (58.4%). InP. aeruginosa–positiveversus –negative patients, lung function was lower, with greater long-term macrolide (21.5%versus14.0%) and inhaled corticosteroid use (63.5%versus53.9%). There was wide regional variation in long-term antibiotic use in patients with bronchiectasis andP. aeruginosa.ASPEN baseline characteristics and treatment profiles were representative of a global bronchiectasis population.

Bronchiectasis Severity Index and FACED scores in patients with allergic bronchopulmonary aspergillosis complicating asthma: do they correlate with immunological severity or high-attenuation mucus?

ABSTRACT Background: The utility of two disease-severity indices, namely bronchiectasis severity index (BSI) and FACED score in allergic bronchopulmonary aspergillosis (ABPA) remains unknown. Objective: To correlate the BSI and FACED scores with immunological parameters (serum IgE [total and A. fumigatus-specific], A. fumigatus-specific IgG, blood eosinophil count), and high-attenuation mucus on chest computed tomography in ABPA. The secondary objectives were to evaluate the correlation between BSI and FACED scores and correlate the BSI/FACED scores with the bronchiectasis health questionnaire (BHQ) and Saint George’s Respiratory Questionnaire (SGRQ). Methods: We included treatment-naïve ABPA subjects with bronchiectasis in a prospective observational study. We computed the BSI and FACED scores for each subject before initiating treatment. The subjects also completed two quality-of-life questionnaires (BHQ and SGRQ). Results: We included 91 subjects. The mean (standard deviation) BSI and FACED scores were 3.43 (3.39) and 1.43 (1.27). We found no correlation between BSI or FACED with any immunological parameter or high-attenuation mucus. There was a strong correlation between BSI and FACED scores (r = 0.76, p < 0.001). We found a weak correlation between BSI and BHQ/SGRQ and FACED and SGRQ. Conclusion: We found no correlation between BSI and FACED with immunological parameters in ABPA. However, we found a significant correlation between BSI and FACED and a weak correlation between SGRQ and BHQ. ABPA likely requires a separate disease-severity scoring system.

Clinical determinants of the modified incremental step test in adults with non-cystic fibrosis bronchiectasis.

This study primarily aimed to investigate the clinical determinants of the Modified Incremental Step Test (MIST) in adults with non-cystic fibrosis bronchiectasis (NCFB). A secondary objective was to compare the cardiopulmonary responses after the MIST and Incremental Shuttle Walk Test (ISWT), two commonly adopted symptom-limited maximum field tests in chronic respiratory diseases. Forty-six patients with clinically stable bronchiectasis participated in this cross-sectional study. MIST and ISWT were performed to determine exercise capacity, while disease severity, fatigue, and quality of life were assessed using the Bronchiectasis Severity Index (BSI), the Fatigue Severity Scale (FSS), and St. George's Respiratory Questionnaire (SGRQ), respectively. Quadriceps muscle strength was evaluated using a hand-held dynamometer, walking speed with a wireless inertial sensing device, and the level of physical activity (steps/day) with a pedometer. The BSI score, quadriceps muscle strength, daily step count, and the SGRQ total score explained 61.9% of the variance in the MIST (p < 0.001, R2 = 0.67, AR2 = 0.619). The BSI score (r = -0.412, p = 0.004), quadriceps muscle strength (r = 0.574, p = 0.001), daily step count (r = 0.523, p < 0.001), walking speed (r = 0.402, p = 0.006), FSS score (r = -0.551, p < 0.001), and SGRQ total score (r = -0.570, p < 0.001) correlated with the MIST. The patients achieved higher heart rates (HR), HR%, desaturation, dyspnea, and leg fatigue in the MIST compared to the ISWT (p < 0.05). Disease severity, quadriceps muscle strength, physical activity level, and quality of life were determinants of MIST. The advantages of the MIST, including higher cardiopulmonary response than ISWT and greater portability, which facilitates its use in various settings, make MIST the preferred choice for investigating symptom-limited exercise capacity in patients with NCFB.

Assessment of Bronchiectasis Severity: The FACED Score versus The Bronchiectasis Severity Index (BSI)

Background: Bronchiectasis is a multi-dimensional, chronic inflammatory and heterogeneous lung disorder characterized by unpredictable clinical course and progression. Two multivariable score systems, the FACED score and the BSI, which are composites of multiple variables have been used to assess the severity and prognosis of bronchiectasis. Objectives: (1) To assess the severity of bronchiectasis in patients using two different validated scores, the FACED score and the Bronchiectasis Severity Index (BSI). (2) To identify microbial profile among bronchiectasis patients and its impact on predicting future exacerbation and hospitalization. Methods: A total of 37 patients from June 2019 to November 2019 were enrolled in this prospective study. The FACED score and BSI score of patients were calculated. Severity of bronchiectasis has been defined by both scores and its impact on exacerbation was examined. Results: Mean age of patients was 45.8 ± 12.7 years. We found mild, moderate and severe bronchiectasis in 17 (45.95%), 15 (40.54%) and 5 (13.51%) patients as assessed by FACED scores. Low, intermediate and high BSI scores were found in 7 (18.92%), 9 (24.32%) and 21 (56.76 %) patients respectively. Patients with high BSI score demonstrated more exacerbations during the follow up period as compared to those with high FACED score. Conclusions: The BSI score is superior to predicting the severity of bronchiectasis as compared to the FACED score. It also helps to identify patients at risk of future exacerbations and hospitalization. Further large-scale studies are recommended to substantiate the findings. Keywords: Bronchiectasis, FACED, BSI, Pseudomonas, Exacerbations

Etiology and clinical characteristics of a non-cystic fibrosis bronchiectasis cohort in a middle eastern population

BackgroundBronchiectasis is a widely prevalent airway disease characterized by airway dilatation and recurrent infections, that can lead to respiratory failure in severe cases. The etiology of bronchiectasis varies geographically, but there is a lack of published data examining its etiology specifically within the Middle Eastern population.MethodsWe conducted a retrospective analysis of our bronchiectasis patient registry, extracting clinical and demographic characteristics from electronic medical records. Quantitative variables were presented as the median and interquartile range (IQR), while categorical variables were expressed as numbers and percentages. Statistical comparisons for continuous characteristics were performed using the t-test, and significance was determined by a p-value less than 0.05.ResultsIn total we analysed 260 records (63% female, 37% male), with median age of 58 years (interquartile range (IQR) 38–71), Body Mass Index (BMI) 25.8(IQR 22–30), forced expiratory volume in the first second (FEV1) %predicted 65 (IQR 43–79) and FEV1/forced vital capacity (FVC) 0.76 (0.67–0.86). Sixty-five cases (25%) were post-infectious in aetiology (excluding post-TB – n:27 10.4%). Forty-eight (18.5%) patients were labelled idiopathic, while Primary Ciliary Dyskinesia (PCD) accounted for 23 (8.8%) cases. Pseudomonas aeruginosa was the most common colonizing organism (32.7%), followed by Haemophilus influenzae (9.2%) and Methicillin-Sensitive Staphylococcus aureus(6.9%). At the time of review, 11 patients had died (median age, FEV %predicted, and bronchiectasis severity index (BSI) 59 years, 38% and 15.5 respectively), all due to respiratory failure, and as expected, all were classed severe on BSI. The BSI score was available for 109 patients, of which 31(28%) were classed mild, 29(27%) were moderate, and 49 (45%) were classed severe. The median BSI score was 8 (IQR 4–11). On dividing the patients according to obstructive vs. restrictive spirometry, we found that patients with FEV1/FVC < 0.70 had significantly higher BSI (10.1 vs. 6.9, p-value < 0.001) and that 8 out of the 11 deceased patients had FEV1/FVC < 70%.ConclusionsIn our study, post-infectious, idiopathic, and PCD were identified as the most common etiologies of bronchiectasis. Additionally, patients with obstructive spirometry appeared to have a worse prognosis compared to those with restrictive spirometry.

Presence and sequence of bronchiectasis onset impact on the clinical characteristics in asthmatic patients.

Asthmatic patients with comorbid bronchiectasis (ACB) show significantly severe condition with various inflammatory phenotypes; bronchiectasis is a heterogeneous disease caused by asthma and other multiple etiological factors. We aimed to investigate the inflammatory characteristics and their clinical significance in asthmatic patients according to the presence and onset time of bronchiectasis. This prospective cohort study recruited outpatients with stable asthma. All the enrolled patients were divided into the non-bronchiectasis group and the ACB group, and the ACB group was separated into the bronchiectasis-prior group and the asthma-prior group. Demographic and clinical data were collected, and peripheral blood and induced sputum eosinophil counts, sputum pathogens, the fraction of exhaled nitric oxide (FeNO), lung function, and chest high-resolution computed tomography were examined. A total of 602 patients (mean age: 55.36±14.58 years) were included, of which 255 (42.4%) were males. Bronchiectasis was present in 268 (44.5%) patients, with 171 (28.41%) in the asthma-prior group and 97 (16.11%) in the bronchiectasis-prior group. For the asthma-prior group, the presence of bronchiectasis was positively correlated with age, presence of nasal polyps, severe asthma, ≥1 pneumonia in the last 12 months, ≥1 severe exacerbation of asthma in the last 12 months (SEA), peripheral blood eosinophil counts, and sputum eosinophil ratio; the extent and severity of bronchiectasis were positively correlated with ≥1 SEA and FeNO levels; and the bronchiectasis severity index (BSI) scores were positively correlated with ≥1 SEA and immunoglobulin E levels. For the bronchiectasis-prior group, bronchiectasis was positively correlated with previous pulmonary tuberculosis or pneumonia in childhood and ≥1 pneumonia in the last 12 months and negatively correlated with forced expiratory volume in one second (FEV1) % and the FeNO level. The extent and severity of bronchiectasis were positively correlated with ≥1 pneumonia in the last 12 months and negatively correlated with FEV1%. The BSI scores were positively correlated with the duration of bronchiectasis. The sequence of bronchiectasis onset may indicate distinct inflammatory characteristics and may be helpful in targeted therapy for patients with asthma.

Features of osteoporosis in male patients with bronchiectasis, a cross-sectional study.

Osteoporosis increases the burden and disease related adverse events of comorbidities in some chronic disease. The relationships between osteoporosis and bronchiectasis are not fully understood. This cross-sectional study explores the features of osteoporosis in male patients with bronchiectasis. From January 2017 to December 2019, male patients (age >50 years) with stable bronchiectasis were included, as were normal subjects. Data on demographic characteristics and clinical features were collected. Totally, 108 male patients with bronchiectasis and 56 controls were analyzed. Osteoporosis was observed in 31.5% (34/108) of patients with bronchiectasis and 17.9% (10/56) of controls (P=0.001). The T-score negatively correlated with age (R=-0.235, P=0.014) and bronchiectasis severity index score (BSI; R=-0.336, P<0.001). BSI score ≥9 was a major factor associated with osteoporosis [odd ratio (OR) =4.52; 95% confidence interval (CI): 1.57-12.96; P=0.005]. Other factors associated with osteoporosis included body-mass index (BMI) <18.5 kg/m2 (OR =3.44; 95% CI: 1.13-10.46; P=0.030), age ≥65 years (OR =2.87; 95% CI: 1.01-7.55; P=0.033), and a smoking history (OR =2.78; 95% CI: 1.04-7.47; P=0.042). The prevalence of osteoporosis was higher in male bronchiectasis patients than that in controls. Factors including age, BMI, smoking history, and BSI were associated with osteoporosis. Early diagnosis and treatment might be of great value in prevention and management of osteoporosis in patients with bronchiectasis.

The Study of Correlation Between Bronchiectasis Severity Index and FACED Score for Assessment of Severity of Bronchiectasis

Introduction: Two different validated scores are currently used to assess the severity of bronchiectasis: the FACED score and the Bronchiectasis Severity Index (BSI). The study was conducted to evaluate clinical etiology in bronchiectasis patients. And to compare the results of the assessment of bronchiectasis severity obtained via FACED and BSI scores. Methods: The study was conducted at a tertiary care hospital in the outpatients of the department of respiratory medicine. Detailed clinical history and necessary investigations were done. BSI and the FACED score were calculated. Statistical analysis was performed using the SPSS package. Results: According to the FACED score, we found 28 patients with mild bronchiectasis, 17 with moderate, and 5 with severe bronchiectasis. The frequency of patients with low, intermediate, and high BSI was 24, 21, and 5, respectively. Moreover, we observed a weak but statistically significant association of 43% agreement between FACED and BSI scores: Fisher’s exact test(p=0.399), tau-b de Kendall (-0.123; p = 0.337) and kappa test (0.032; p = 0.878). Conclusions: There is a small but significant correlation between the two scales (BSI and FACED). a tendency is observed for patients to be classified with a higher BSI compared to the FACED score.

Improving care for people with bronchiectasis: opportunities and challenges highlighted from service evaluation.

Bronchiectasis is a serious, debilitating condition warranting specialist care. To determine if care provided in a tertiary hospital general respiratory clinic was guideline concordant and to validate the Bronchiectasis Severity Index (BSI) in the Australian context. A single-centre ambispective study was conducted. The first stage involved a retrospective medical record audit between 1 January 2015 and 31 December 2016. All aspects of bronchiectasis management were reviewed. In the second prospective phase the cohort was followed for 4 years to determine survival and the validity of the BSI determined. A total of 145 patients was included, with mean age of 65 years (standard deviation=16.6). The aetiology of bronchiectasis was explicitly documented for 58 (40%) patients, with potential causes identified in another 37 patients. Post-infectious aetiologies were described in 62 (43%) patients. Most patients had lung function testing (n = 142; 97%) and sputum culture results (n = 120; 83%). Long-term antibiotics were prescribed to 49 (34%) patients. Only patients culturing Pseudomonas spp. were prescribed inhaled antibiotics. Documentation regarding essential management recommendations was low, including airway clearance (46%), pneumococcal vaccination (27%) and written action plans (32%). Severe disease was common, with more than one-third (34-48%) having BSI scores >9. One-fifth (21%) of the cohort died during the 4-year follow-up period. The BSI was significantly associated with mortality risk (odds ratio 7.7; 95% confidence interval=3.1-19.3; P < 0.001). Our cohort had a high proportion of patients with severe disease and significant mortality; some, but not all, aspects of recommended care were delivered.

Serum Fibrinogen as a Biomarker for Disease Severity and Exacerbation in Patients with Non-Cystic Fibrosis Bronchiectasis.

Background: Serum biomarkers associated with severe non-cystic fibrosis (CF) bronchiectasis are currently lacking. We assessed the association of serum fibrinogen, adiponectin, and angiopoietin-2 levels with the severity and exacerbation of bronchiectasis. Methods: Serum levels of fibrinogen, adiponectin, and angiopoietin-2 were measured and compared in patients with stable non-CF bronchiectasis (n = 61) and healthy controls (n = 16). The correlations between the three biomarkers and the bronchiectasis severity index (BSI) or FACED scores were assessed. Univariate and multivariate linear regression analyses were performed to identify variables independently associated with BSI and FACED scores in patients with bronchiectasis. Additionally, the exacerbation-free survival was compared between groups of patients with high and low fibrinogen levels, while the predictors of exacerbation were analyzed using Cox proportional hazards regression. Results: Patients with non-CF bronchiectasis carried higher fibrinogen (3.00 ± 2.31 vs. 1.52 ± 0.74 µg/mL; p = 0.016) and adiponectin (12.3 ± 5.07 vs. 9.17 ± 5.30 µg/mL; p = 0.031) levels compared with healthy controls. The serum level of angiopoietin-2 was comparable between the two groups (1.49 ± 0.96 vs. 1.21 ± 0.79 ng/mL, p = 0.277). Correlations of adiponectin and angiopoietin-2 with BSI and FACED scores were not significant. However, there were significant correlations between fibrinogen and both BSI (r = 0.428) and FACED scores (r = 0.484). Multivariate linear regression analysis revealed that fibrinogen level was an independent variable associated with both BSI and FACED scores. A total of 31 (50.8%) out of 61 patients experienced exacerbation during the follow-up period of 25.4 months. Exacerbation-free survival was significantly longer in patients with low fibrinogen levels than in those with high fibrinogen (log-rank test, p = 0.034). High fibrinogen levels and Pseudomonas colonization were independent risk factors for future exacerbation (HR 2.308; p = 0.03 and HR 2.555; p = 0.02, respectively). Conclusions: Serum fibrinogen, but not adiponectin or angiopoietin-2, is a potential biomarker closely associated with the severity and exacerbation of non-CF bronchiectasis.

Correlation among clinical, functional and morphological indexes of the respiratory system in non-cystic fibrosis bronchiectasis patients.

BackgroundNon-cystic fibrosis bronchiectasis (NCFB) is a heterogeneous disease, which assessment and severity can’t be defined by one particular instrument but using a multidimensional score. Thus, in additional to traditional methods, alternative tools have been developed to assist these patients’ evaluation.ObjectiveTo correlate functional and morphological indexes with severity and dyspnea in NCFB patients, focusing on the correlation between the impulse oscillometry system (IOS) and the quantitative analysis of computed tomography (CT).MethodsClinically stable NCFB patients, between 18 and 80 years old were submitted to clinical, functional and morphological evaluations assessed by Bronchiectasis Severity Index (BSI) and Medical Research Council (MRC) scale; spirometry and IOS; and subjective and quantitative Chest CT scans analysis, respectively.ResultsThis study included 38 patients. The best correlations obtained between functional and morphological airway indexes were: resistance at 5 Hz—R5 and the normalized thickness of bronchial walls—Pi10 (r = 0.57), and the mean forced expiratory flow (FEF25-75%) and CT score (r = -0.39). BSI as well as MRC showed higher correlations with the quantitative automated analysis of CT (BSI and Pi10: r = 0.41; MRC and Pi10: r = 0.35) than with subjective CT score (BSI and CT score: r = 0.41; MRC and CT score: r = 0.15); and moderate and weak correlations were obtained on both functional airway indexes (BSI and peripheral airways resistance - R5-R20: r = 0.53; BSI and forced expiratory volume at the first second—FEV1: R = -0,64; MRC and R5-R20: r = 0.42; and MRC and VEF1: r = -0.45).ConclusionIn NCFB patients, compartmentalized methods for assessing the respiratory system (IOS and the automated quantitative CT analysis) have a good correlation with severity and dyspnea.

In bronchiectasis, poor physical capacity correlates with poor quality of life

ABSTRACT Purpose Patients with bronchiectasis (BE) who suffer frequent exacerbations are likely to experience negative effects on quality of life (QoL) and require more healthcare utilization. We aimed to discover, in a cohort of Finnish BE patients, those risk factors that influence QoL. Methods Non-cystic fibrosis BE patients of a Helsinki University Hospital cohort were examined with high-resolution computed tomography (HRCT) of the chest. They completed a disease-specific quality of life-bronchiectasis (QoL-B) questionnaire in Finnish translation. We considered scores in the lowest quarter (25%) of that QoL-B scale to indicate poor QoL. The bronchiectasis severity index (BSI), FACED score, and modified Medical Research Council (mMRC) dyspnoea scale were used. Results Overall, of 95 adult BE patients, mean age was 69 (SD ± 13) and 79% were women. From the cohort, 82% presented with chronic sputum production and exacerbations, at a median rate of 1.7 (SD ± 1.6). The number of exacerbations (OR 1.7), frequent exacerbations (≥3 per year) (OR 4.9), high BSI score (OR 1.3), and extensive disease (≥3 lobes) (OR 3.7) were all predictive of poor QoL. Frequent exacerbations were associated with bronchial bacterial colonisation, low forced expiratory volume in 1 s (FEV1), and radiological disease severity. Based on the BSI, 34.1% of our cohort had severe disease, with 11.6% classified as severe according to their FACED score. The mMRC dyspnoea score (r = −0.57) and BSI (r = −0.60) correlated, in the QoL-B questionnaire, negatively with physical domain. Conclusion The strongest determinants of poor QoL in the cohort of Finnish BE patients were frequent exacerbations, radiological disease severity, and high BSI score. Neither comorbidities nor BE aetiology appeared to affect QoL. Reduced physical capacity correlated with dyspnoea and severe disease. Study registration University of Helsinki, Faculty of Medicine, 148/16.08.2017.

Sputum neutrophil elastase and its relation to pediatric bronchiectasis severity: A cross-sectional study.

Background and AimsSputum neutrophil elastase (NE) is a marker of neutrophilic airway inflammation in bronchiectasis. Yet, not much is known about its role in pediatric bronchiectasis severity. This study aimed to assess the sputum NE value as a biomarker of clinical and radiological severity in pediatric bronchiectasis.MethodsThis was a cross‐sectional study assessing sputum NE in a total of 50 bronchiectasis patients under the age of 18 years—30 patients with cystic fibrosis (CF) and 20 patients with non‐CF bronchiectasis were included. Bronchiectasis severity was assessed using Shwachman–Kulczycki (SK) score, CF‐ABLE score, and CF risk of disease progression score, among CF patients, and bronchiectasis severity index (BSI) and FACED criteria among non‐CF bronchiectasis patients, associations between sputum NE and bronchiectasis severity were assessed in both patient groups.ResultsSputum NE was directly correlated with C‐reactive protein (r = 0.914, p < 0.001), (r = 0.786, p < 0.001), frequency of exacerbations (r = 0.852, p < 0.001) (r = 0.858, p < 0.001), exacerbations severity (r = 0.735, p = 0.002), (r = 0.907, p < 0.001), and the number of hospital admissions (r = 0.813, p < 0.001), (r = 0.612, p =0.004) in the last year among CF, and non‐CF bronchiectasis patients, respectively. Additional linear correlations were found between sputum NE, CF risk of disease progression score (p < 0.001), CF‐ABLE score (p < 0.001), and lower forced expiratory volume 1% of predicted (p = 0.017; ρ = −0.8) among CF patients. Moreover, sputum NE was positively correlated with the neutrophil count (p = 0.018), and BSI severity score (p = 0.039; ρ = 0.465) among non‐CF bronchiectasis patients.ConclusionsSputum NE may be considered a good biomarker of bronchiectasis severity in both CF and non‐CF bronchiectasis patients, as confirmed by the exacerbations rate, CF risk of disease progression, and BSI scores.

Neutrophil side fluorescence: a new indicator for predicting the severity of patients with bronchiectasis

BackgroundNeutrophilic inflammation in the airway is a hallmark of bronchiectasis. Neutrophil extracellular traps (NETs) have been reported to play an important role in the occurrence and development of bronchiectasis. Neutrophil side fluorescence is one of the characteristics of neutrophils that can reflect the activation of neutrophils and the formation of NETs.ObjectiveTo explore the relationship between the values of neutrophil side fluorescence (NEUT-SFL) in the peripheral blood of bronchiectasis patients, and the severity of the disease.Methods82 patients with bronchiectasis from the Department of Respiratory and Critical Medicine, at the Third Affiliated Hospital of Southern Medical University and were scored with Bronchiectasis Severity Index (BSI) (2019–2021). The clinical data such as the value of NEUT-SFL, neutrophil count, C-reactive protein, and procalcitonin levels were collected and retrospectively analyzed. NEUT-SFL values neutrophil count from 28 healthy subjects were also used to ascertain cut-off values.ResultsBased on the BSI scores, patients were divided into three categories as mild (32%), moderate (29%), and severe (39%). Our results showed that the values of NEUT-SFL were higher in bronchiectasis patients compared to healthy controls. The levels of NEUT-SFL positively correlated with the high BSI scores in patients (P = 0.037, r = 0.23) and negatively correlated with the lung function in these patients (r = − 0.35, P = 0.001). The area under the ROC curve was 0.813, the best cut-off was 42.145, indicating that NEUT-SFL values > 42.145 can potentially predict the severity of bronchiectasis.ConclusionsThe values of NEUT-SFL in the peripheral blood can be used for predicting the severity of bronchiectasis.

Efficacy of pulmonary rehabilitation on patients with non-cystic bronchiectasis according to disease severity.

The European Respiratory Society guidelines support pulmonary rehabilitation (PR) in bronchiectasis through high-quality evidence. This study aimed to evaluate the efficacy of PR on bronchiectasis patients according to disease severity assessed by the Bronchiectasis Severity Index (BSI). This prospective study included patients with stable bronchiectasis. Demographic data of all patients were questioned. All patients underwent an 8-week PR program. The patients were grouped into three according to disease severity (mild, moderate and severe) based on their BSI scores. The following parameters were evaluated at baseline (pre-PR) and after PR (post-PR): pulmonary function test results, carbon monoxide diffusion capacity, body mass index, exercise capacity (6-minute walking test), quality of life (QoL; St. George's Respiratory Questionnaire), and Hospital Anxiety and Depression scores. The study included 69 patients (55 males; mean age, 62.6 ± 9 years). After PR, the patients were observed to have significantly improved QoL and exercise capacity (p< 0.05). According to BSI, 16 (23.2%), 29 (42.0%), and 24 (34.8%) patients had mild, moderate, and severe bronchiectasis, respectively. These patient groups significantly differed regarding age, exercise capacity, and QoL (p< 0.05). Comparing the change between post-PR and pre-PR values of the study parameters (∆ = post-PR value - pre-PR value), no significant differences were observed regarding the exercise capacity and QoL (p> 0.05) in the groups. PR increases exercise capacity and QoL in bronchiectasis patients, and its efficacy does not differ according to disease severity. Thus, appropriate bronchiectasis patients should be referred to PR program regardless of disease severity.

Type 2-High Severe Asthma with and without Bronchiectasis: A Prospective Observational Multicentre Study.

IntroductionType 2-high severe asthma (T2-SA) is often associated with several comorbidities. To this extent, the coexistence of T2-SA and bronchiectasis (BE) is considered an emerging phenotype.MethodsWe performed a prospective observational multicentre study, including T2-SA patients. Chest HRCT confirmed the presence of BE. Data on exacerbations, pulmonary function, Asthma Control Test (ACT), chronic mucus hypersecretion (CMH), chronic rhinosinusitis (CRS), oral corticosteroid (OCS) dosage, eosinophils in peripheral blood and FeNO were recorded. The Bhalla score was used for radiological assessment of T2-SA+BE patients and the Bronchiectasis Severity Index (BSI) was calculated.ResultsA total of 113 patients (mean age 55 ± 11 years, 59.3% female) were enrolled. Co-presence of BE was confirmed in 50/113 (44.2%) patients who identified the T2-SA+BE group. CRS and CRSwNP were more prevalent in T2-SA+BE vs T2-SA [respectively, 42/50 (84%) vs 37/63 (58.7%), p = 0.004 and 27/50 (54%) vs 27/63 (42.9%), p = 0.0165]. Furthermore, T2-SA+BE patients reported more CMH compared to T2-SA [29/50 (58%) vs 15/63 (23.8%), p = 0.0004], were more frequently on chronic OCSs intake [28/50 (56%) vs 22/63 (34.9%), p = 0.0357] and experienced more exacerbations/year [10 (4–12) vs 6 (4–12), p = 0.0487]. In a multivariate logistic regression model, the presence of CRS, CMH and daily OCS intake were associated with BE presence with a 78% (95% CI: 69–88) accuracy. Median Bhalla score was 18.3 (16–20) (Mild radiological severity). Median BSI was 6 (4–8) and only 6/50 (12%) had a BSI score ≥9. Significant inverse linear relationship between BSI and ACT (r = −0.6095, p < 0.0001), FEV1% (r = −0.3297, p = 0.0353) and FEV1 mL (r = −0.4339, p = 0.0046) were found.ConclusionType 2 inflammation could have a causative role in BE development. Chest HRCT is mandatory when a diagnosis of T2-SA is made, especially in presence of CRS, CMH and chronic OCS intake. Early BE detection may be crucial to improve T2-SA patients’ outcomes.

Heterogeneity of non-cystic-fibrosis bronchiectasis in multiethnic Singapore: A prospective cohort study at a tertiary pulmonology centre

Non-cystic fibrosis bronchiectasis (NCFB) is a highly heterogenous disease. We describe the clinical characteristics of NCFB patients and evaluate the performance of Bronchiectasis Severity Index (BSI) in predicting mortality. Patients attending the bronchiectasis clinic between August 2015 and April 2020 with radiologically proven bronchiectasis on computed tomography were recruited. Clinical characteristics, spirometry, radiology, microbiology and clinical course over a median period of 2.4 years is presented. A total of 168 patients were enrolled in this prospective cohort study. They were predominantly women (67.8%), Chinese (87.5%) and never-smokers (76.9%). Median age of diagnosis was 64 years (interquartile range 56-71) and the most common aetiology was "idiopathic" bronchiectasis (44.6%). Thirty-nine percent had normal spirometries. Compared to female patients, there were more smokers among the male patients (53.8% versus 8.5%, P<0.001) and a significantly larger proportion with post-tuberculous bronchiectasis (37.0% vs 15.8%, P=0.002). Fifty-five percent of our cohort had a history of haemoptysis. Lower body mass index, presence of chronic obstructive pulmonary disease, ever-smoker status, modified Reiff score, radiological severity and history of exacerbations were risk factors for mortality. Survival was significantly shorter in patients with severe bronchiectasis (BSI>9) compared to those with mild or moderate disease (BSI<9). The hazard ratio for severe disease (BSI>9) compared to mild disease (BSI 0-4) was 14.8 (confidence interval 1.929-114.235, P=0.01). The NCFB cohort in Singapore has unique characteristics with sex differences. Over half the patients had a history of haemoptysis. The BSI score is a useful predictor of mortality in our population.

Which scoring system is better in association with exercise capacity and health status in noncystic fibrosis bronchiectasis patients?

Background/aim Two different scoring systems were developed to determine the severity of bronchiectasis: FACED scoring and the bronchiectasis severity index (BSI). In this study, we aim to compare these 2 scoring systems according to the 6-min walking distance test and a disease-specific health status questionnaire in patients with noncystic fibrosis bronchiectasis (NCFB). Materials and methods Smoking history, emergency and hospital admissions, and body mass index were obtained from NCFB patients admitted to our hospitals’ pulmonary rehabilitation unit between 2013 and 2018. Detailed pulmonary function tests were performed for all participants. Dyspnea perceptions were determined according to the mMRC dyspnea scale. The 6-min walking test was used to determine exercise capacity. The Saint George respiratory questionnaire (SGRQ) was applied to determine health status. Both FACED and BSI scores were calculated for all participants. Results There were a total of 183 participants, 153 of whom were men. A significant and strong correlation was found between FACED and BSI scores. As the severity of bronchiectasis increased, walking distance was significantly decreased and health status was significantly worse in both FACED and BSI scoring. A statistically significant but weak negative correlation was found between FACED score and walking distance. There was a significant negative correlation between BSI and walking distance, a stronger negative correlation than with FACED. Similarly, there was a significant negative correlation between health status and both FACED and BSI, but this correlation was stronger in the BSI score. Conclusion Although both FACED and BSI scores were negatively correlated with walking distance and health status in patients with NCFB, BSI was more strongly associated.

Primary Immun Deficiency in Patients with Non-Cystic Fibrosis Bronchiectasis and Its Relationship with Clinical Parameters.

Bronchiectasis is characterized by chronic respiratory infection. The role of immunodeficiency in this disease is poorly studied in relation to clinical indices. The primary aim of this study was to determine the frequency of these neglected altered immune status by evaluating immunoglobulins, lymphocyte subsets, complement levels, and neutrophil function, and to assess its relationship with clinical parameters in adult patients with non-cystic fibrosis bronchiectasis (NCFB). A total of 74 (30 men and 44 women with a mean age of 47±17 years) adult patients with stable NCFB were enrolled in this study. The bronchiectasis severity index (BSI) and FACED (F:FEV1, A: Age, C: Chronic colonization, E: Extension, D: Dyspnea) scores were assessed. Peripheral blood samples were collected for the detection of total IgG, IgA, IgM, IgE, and IgG subclasses and C3 and C4 levels. The counts of CD3, CD4, CD8, CD19, CD16/56 expressing peripheral blood lymphocytes and neutrophil oxidative function were evaluated. In the study population, BSI and FACED severity index scores increased with longer duration of the disease (p=0.01 and p=0.040, respectively). Of the 74 patients, 27 (37%) showed humoral aberrations. The number of male patients were higher in this group (p=0.03). High serum total IgE levels were associated with high scores in BSI (moderate-severe group versus mild group, p=0.030). Patients with bronchiectasis demonstrated lower CD3+ T cell count, lower CD4+ T helper cell percentage, and lower CD4+ T cell count (p=0.031, p=0.030, p=0.029, respectively) than healthy subjects. A significant negative correlation was found between the percentage and count of CD16/56+ natural killer (NK) cells and the number of exacerbations within the past year (r=-0.230, p=0.049 and r=-0.264, p=0.023, respectively). Humoral aberrations in adult patients with NCFB were found to be frequent. IgE levels were related to high scores for disease severity indices. Furthermore, patients with low percentage and counts of NK cells had higher rates of exacerbations. These results emphasize the importance of immune function assessment in adult patients with NCFB.

Predicting mortality in non-cystic fibrosis bronchiectasis patients using distance-saturation product

Background The bronchiectasis severity index (BSI) and FACED score are currently used in predicting outcomes of non-cystic fibrosis bronchiectasis (NCFB). Distance-saturation product (DSP), the product of distance walked, and lowest oxygen saturation during the 6-min walk test showed strong predictive power of mortality in non-CF bronchiectasis patients. This study aimed to compare the efficacy of these scores and DSP in predicting mortality. Methods and Patients Our retrospective study included NCFB patients from January 2004 to December 2017. We recorded the basic data, pulmonary function, radiologic studies, sputum culture results, acute exacerbations (AE), emergency department (ED) visits, hospitalization, and mortality. Results A total 130 NCFB patients were analysed. The mean BSI score, FACED score, and DSP were 8.8 ± 4.9, 3.4 ± 1.7, and 413.1 ± 101.5 m%, respectively. BSI and FACED scores had comparable predictive power for AE (p=.011; p=.010, respectively). The BSI score demonstrated a significant correlation with ED visits (p=.0003). There were 12 deaths. Patients were stratified using a DSP cut-off value of 345 m% according to the best area under receiver operator characteristic curve (AUC) value in mortality. DSP was not correlated with AE and ED visits. BSI, FACED scores, and DSP demonstrated statistically significant correlations with hospitalization (p<.0001; p<.0001; p=.0007, respectively). The AUC for overall mortality was similar for BSI, FACED score, and DSP (0.80 versus 0.85, p=.491; 0.85 versus 0.83, p=.831). Conclusion DSP had comparable predictive power for mortality as the well-validated BSI and FACED scores and is relatively easy to use in clinical practice. KEY MESSAGE Distance-saturation product (DSP) comprised with the product of distance walked, and lowest oxygen saturation during the 6-min walk test, which is common used in clinical practice. DSP demonstrated strong and comparable predictive power of mortality as the well-validated BSI and FACED scores in non-CF bronchiectasis patients.