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PubMed2005

Non-CF bronchiectasis: does knowing the aetiology lead to changes in management?

The aim of the current study was to review the aetiology of non-cystic fibrosis (CF) bronchiectasis from two tertiary paediatric respiratory units in order to determine how often making a specific aetiological diagnosis leads to a change in management, and to assess the contribution of computed tomography (CT) in determining the underlying diagnosis. The case records of all patients who were diagnosed as having bronchiectasis by CT, currently being seen at the Royal Brompton Hospital and Great Ormond Street Hospital for Children (London, UK), were reviewed. All patients had undergone extensive investigations, and the underlying aetiology and the area of pulmonary involvement (as seen on CT) were recorded. A total of 136 patients were identified; there were 65 young males and the group median (range) age was 12.1 yrs (3.1-18.1). Immunodeficiency, aspiration and primary ciliary dyskinesia accounted for 67% of the cases. In 77 (56%) children, the identification of a cause led to a specific change in management. There was no association between aetiology and the distribution of CT abnormalities. In conclusion, immunodeficiency and other intrinsic abnormalities account for the majority of cases of non-cystic fibrosis bronchiectasis seen in the current authors' units. Computed tomography scans do not contribute towards identifying the aetiology and, most importantly, a specific aetiological diagnosis frequently leads to a change in management.

Adolescent↗

PubMed1997

Bronchiectasis: an orphan disease with a poorly-understood prognosis.

The prognosis and risk factors for bronchiectasis are at present poorly known. The aim of this study was to examine the long-term prognosis and cause of death in this disease. The National Hospital Discharge Register was used to search for patients aged 35-74 yrs, with newly-diagnosed bronchiectasis in the period 1982-1986. Each of the 842 patients identified was matched with an asthmatic patient and a patient with chronic obstructive pulmonary disease (COPD), who were of the same age and sex and who had been treated in hospital at the same time. The use of hospital services by these subjects was examined up to the end of 1992, and mortality to the end of 1993. The prognosis for the bronchiectatic patients treated in hospital was better than that for the COPD patients but poorer than that for the asthmatics; the risk of death being 1.25 (95% confidence interval (95% CI) 1.15-1.36) for the COPD patients and 0.79 (95% CI 0.71-0.87) for the asthmatics, relative to the bronchiectatic patients. Bronchiectasis was the main cause of death in 13% of bronchiectatic patients, the risk of death being increased by a factor of 1.21 in the presence of asthma as the main secondary diagnosis, by 1.31 with COPD, by 1.35 with tuberculosis and its sequelae, and by 1.32 with some other secondary diagnosis, as compared with cases for which no secondary diagnosis was indicated. The fact that the prognosis for bronchiectatic patients is poorer than that for asthmatics points to a continued need for focused care and follow-up, particularly in the presence of additional illnesses.

Adult↗

PubMed1998

Systemic markers of inflammation in stable bronchiectasis.

Patients with bronchiectasis have an active local and systemic inflammatory response during infective exacerbations. Systemic markers of inflammation were investigated during a stable phase of their illness, because continued inflammation could affect their general health and be involved in disease progression. The relationship between levels of various systemic markers of inflammation and extent of disease on computed tomographic scan, lung function, sputum bacteriology and health related quality of life (HRQoL) was investigated in 87 noncystic fibrosis bronchiectasis patients. Several markers were elevated and correlated with the extent of disease and poor lung function. The total white cell count, neutrophil count and erythrocyte sedimentation rate correlated with both disease measures. Sputum bacteriology did not correlate with inflammation markers and patients with positive and negative cultures were similar. C-reactive protein and total white cell count correlated with some components of a disease-specific HRQoL questionnaire. In conclusion, patients with bronchiectasis in a stable phase have raised systemic markers of inflammation. Some markers, particularly the neutrophil count, correlate with disease severity. This result is in keeping with the hypothesis that the level of inflammation determines disease progression and health status.

Adult↗

PubMed1998

Exhaled nitric oxide is not elevated in the inflammatory airways diseases of cystic fibrosis and bronchiectasis.

Airways inflammation has been associated with increased nitric oxide (NO) in the exhaled breath. It was, therefore, questioned whether exhaled NO could act as an indicator of the severity of airways inflammation in the chronic suppurative lung diseases cystic fibrosis (CF) and bronchiectasis. NO levels in a single exhalation were measured using a chemiluminescence analyser. Thirty-six patients with CF and 16 with bronchiectasis were studied and compared with 22 normal subjects and 35 asthmatic patients. All subjects were nonsmokers and all measurements were made when patients were clinically stable. In addition, exhaled NO was measured in 10 CF patients at the time of onset of an acute infective exacerbation and followed for 7 days during the treatment of the exacerbation in eight of the 10 patients. No significant differences were found in NO levels in patients with CF or bronchiectasis compared with normals (median 4.0, 5.5 and 4.4 parts per billion (ppb), respectively), but all were lower than in asthma patients (10.4 ppb). The NO levels in the CF patients at time of exacerbation were not increased and did not change during treatment. These data show that nitric oxide levels in the exhaled breath of patients with chronic suppurative lung diseases, in contrast to asthma, are not elevated, despite the presence of substantial airways inflammation. Possible explanations include poor diffusion of nitric oxide across increased and viscous airway secretions, removal of nitric oxide by reaction with reactive oxygen species in the inflamed environment and failure of upregulation of epithelial inducible nitric oxide synthase in chronic suppurative conditions.

Adult↗

PubMed1999

A comparative study on the efficacy of levofloxacin and ceftazidime in acute exacerbation of bronchiectasis.

A prospective randomized study was performed in order to compare the efficacy of oral levofloxacin, a new S- isomer of ofloxacin, with intravenous ceftazidime in the empirical treatment of acute exacerbations of bronchiectasis. Consecutive patients with acute exacerbation of bronchiectasis were recruited at a tertiary referral centre and were randomized to receive 10 days' treatment with either oral levofloxacin (300 mg b..d.) or ceftazidime (1 g i.v.t.d.). Body temperature, cough score, dyspnoea score, sputum purulence and volume and white blood cell and neutrophil count were assessed on day 1 and day 10. Thirty-five patients (mean age 61 yrs, 15 males) completed the study; 17 of these were in the levofloxacin group. There was no significant difference in the distribution of sputum pathogens or clinical parameters between the two groups at entry to and completion of the study. Both groups of patients showed significant improvement in 24-h sputum volume, sputum purulence score, cough score and dyspnoea score (p<0.001) but there was no significant difference between these two groups at entry to or on completion of the study (p>0.05). The results of this study suggest that oral administration of levofloxacin is as effective as parenteral ceftazidime in the empirical treatment of exacerbations in bronchiectasis.

Acute Disease↗

PubMed2026

Bridging the airway microbiome and targeted therapy in bronchiectasis: multi-omics insights, endotypes and emerging therapies.

Bronchiectasis is a heterogeneous chronic airway disease primarily driven by persistent infection, microbial dysbiosis and dysregulated host immunity. While culture-based microbiology has historically informed clinical management, advances in high-throughput sequencing and multi-omic technologies have transformed our understanding of the airway ecosystem, revealing that disease activity is shaped not only by individual pathogens, but by complex and dynamic host-microbe interactions. Despite the breadth of descriptive microbiome data, translation into clinically actionable diagnostics or therapies has been limited. Importantly, cross-sectional correlations between microbiota and inflammation do not establish cause and effect, underscoring the need to embed host-microbiome profiling within both longitudinal and interventional therapeutic trials. In this review, we critically appraise current microbial and host multi-omics research in bronchiectasis, integrating microbiome studies with host inflammatory, proteomic and immunophenotyping data. We highlight themes emerging across cohorts, including low microbial diversity, pathogen dominance, loss of commensal networks and neutrophil-driven inflammation, and discuss how these features align with biological endotypes associated with exacerbations and treatment response. Drawing on lessons from host-directed therapeutic successes, we examine translational roadblocks limiting microbiome-guided care. We further review emerging microbiome-modulating strategies such as pathogen-specific biologics, bacteriophage therapy, live biotherapeutic products, biofilm-targeting adjuncts and precision antibiotic stewardship. Finally, we propose a roadmap toward microbiome-informed precision medicine through harmonised methodologies, integration of host and microbial biomarkers into clinical trials, and embedding multi-omics pipelines within large international registries. Collectively, these advances have the potential to shift bronchiectasis research and clinical management towards rationally designed, precision medicine-driven therapeutic strategies.

Humans↗

PubMed2004

Airway clearance in bronchiectasis: a randomized crossover trial of active cycle of breathing techniques (incorporating postural drainage and vibration) versus test of incremental respiratory endurance.

OBJECTIVES: The purpose of this study was to compare the efficacy of the test of incremental respiratory endurance (TIRE) with active cycle of breathing techniques (ACBT) [incorporating postural drainage (PD) and vibration] as methods of airway clearance in adults with bronchiectasis. DESIGN: A randomized crossover study in which a single session of ACBT (incorporating PD and vibration) was compared to a single session of TIRE was carried out in 20 patients (14 female) with stable, productive bronchiectasis. MEASUREMENTS: Weight of sputum (treatment plus 30 min) was the primary outcome measure recorded. Pre- and post-treatment measures of lung function and SpO2 were also recorded. RESULTS: All 20 patients were stable during the study period. Sputum weight expectorated during and 30 min post-ACBT (incorporating PD and vibration) treatment was significantly greater than the sputum weight expectorated during and 30 min post-TIRE treatment [mean difference 2.44 g (95% CI 0.43-4.45)]. CONCLUSION: ACBT (incorporating PD and vibration) is a more effective method of airway clearance in bronchiectasis than TIRE during single treatment sessions.

Bronchiectasis↗

PubMed1996

Review article: imaging in bronchiectasis.

Bronchiectasis remains an important and relatively common cause of pulmonary disability. High resolution computed tomography has revolutionized imaging of the bronchi and has superseded the chest radiograph and bronchogram in the diagnosis of bronchiectasis. It has enabled a better understanding of the association between clinical features of the disease and structural abnormalities in the airways and has an important role to play in understanding the pathogenesis and natural history of bronchiectasis.

Bronchiectasis↗

PubMed1993

Bronchiectasis in patients with alpha 1-antitrypsin deficiency. A rare occurrence?

The chest radiographs and computed tomographic (CT) scans of seven patients with homozygous proteinase inhibitor phenotype Z (PiZZ) alpha 1-antitrypsin deficiency were reviewed. All patients except one showed severe emphysema with or without bullous change. Bronchiectasis was detected in three patients by CT but only in two patients by chest radiography. A young patient developed bronchiectasis before symptomatic emphysema. We stress that patients with PiZZ are susceptible to bronchiectasis, and the widespread use of CT should reveal its true incidence which might not be as low as generally believed.

Adult↗

PubMed1994

Computed tomographic diagnosis of Mycobacterium avium-intracellulare complex in patients with bronchiectasis.

We tested the hypothesis that bronchiectasis and multiple small lung nodules seen on chest computed tomography (CT) are indicative of Mycobacterium avium-intracellulare complex (MAC) infection or colonization by reviewing CT scans and histories of 100 outpatients with CT diagnosis of bronchiectasis. Of the 24 patients with multiple pulmonary nodules, 19 had lung nodules and bronchiectasis in the same lobe. Mycobacterial cultures were performed on 63 of the 100 patients, including 15 of the 24 patients with lung nodules and 48 of the 76 patients with no lung nodules. Of the 15 patients with lung nodules, 8 (53 percent) had cultures positive for MAC, as did 2 of the 48 (4 percent) patients with no CT evidence of lung nodules. The number of cultures positive for fungi was approximately the same in both groups. In our outpatient population, CT prediction of cultures positive for MAC in bronchiectatic patients with multiple small lung nodules has a sensitivity of 80 percent, a specificity of 87 percent, and an accuracy of 86 percent.

Adolescent↗

PubMed1994

Gelatinolytic and type IV collagenolytic activity in bronchiectasis.

To evaluate the extracellular matrix (ECM) degradation in bronchiectasis (BE), the level of gelatinolytic and type IV collagenolytic activity was analyzed in bronchoalveolar lavage fluid (BALF) by using zymographies. The BALF of patients with bronchiectasis revealed a high gelatinolytic and type IV collagenolytic activity whereas no such activities were detected in BALF of the healthy controls. Furthermore, the level of degradative activity correlated with the severity of disease with a spectrum varying from patients characterized by frequent pneumonia and bronchitis, mucopurulent and purulent sputum production, and saccular changes of bronchi having high activities of both 92-kd and 72-kd gelatinases type IV collagenases (corresponding to the neutrophil type MMP-9 and fibroblast type MMP-2 activities, respectively) to patients having few clinical symptoms and displaying only a weak activity at the 92-kd area. These findings suggest a role for the matrix metalloproteinases MMP-2 (72-kd gelatinase/type IV collagenase) and MMP-9 (92-kd gelatinase/type IV collagenase) in the degradation of ECM of bronchial wall and lung tissue. In addition, severe bronchiectasis was associated with the presence of low-molecular weight gelatinases reflecting in vivo metalloproteinase activation and/or the presence of microbial-derived gelatinolytic proteinases.

Adolescent↗

PubMed1997

Long-term efficiency of home nasal mask ventilation in patients with diffuse bronchiectasis and severe chronic respiratory failure: a case-control study.

The aim of this study was to evaluate long-term efficacy and tolerance of nasal mask ventilation (NMV) in a comparative case-control study. Fourteen patients with diffuse bronchiectasis and severe chronic respiratory failure (CRF), treated by long-term oxygen-therapy (LTO) and NMV, were case matched with 14 patients with diffuse bronchiectasis and severe CRF treated with only LTO. Patients and control subjects were compared based on the following parameters: blood gases, FEV1, vital capacity, hospitalizations, and survival. Symptoms, Karnofsky function score, and clinical evolution were also monitored in patients. Three subgroups may be identified according to outcome: two early deaths (subgroup 1), six patients with initial improvement and subsequent deterioration (subgroup 2), and six patients whose conditions remained improved for >2 years (subgroup 3). PaO2 decrease slope was slighter in this last subgroup than subgroup 2. The days of hospitalization were significantly reduced after institution of NMV in the patient group. Comparison between patients and control subjects did not show any difference on PaO2 evolution and on the overall median survival (46 and 40 months in NMV and control group, respectively). Long-term tolerance and compliance remained satisfactory for 11 patients. These results suggest that NMV is feasible as a long-term home treatment in patients with diffuse bronchiectasis. Although our results may have failed to prove a long-term efficiency on the course of blood gases and survival, a beneficial effect is observed with reduction of hospitalizations and improvement of functional status. This study warrants further investigation, in a prospective series, with a larger number of patients.

Adult↗

PubMed1998

The effect of Pseudomonas aeruginosa infection on clinical parameters in steady-state bronchiectasis.

STUDY OBJECTIVE: To investigate the effect of Pseudomonas aeruginosa infection on clinical parameters in Chinese patients with noncystic fibrosis and steady-state bronchiectasis. DESIGN: Prospective, cross-sectional clinicomicrobiological study with informed consent. SETTING: Consecutive outpatient recruitment from a specialist bronchiectasis respiratory clinic. PATIENTS: Outpatients (n = 100; 62 women; 55.1+/-16.7 years old; FEV1/FVC 1.4+/-0.7/2.1+/-0.9 L), who had stable respiratory symptoms for more than 3 weeks. MEASUREMENTS AND RESULTS: Respiratory pathogens isolated from the sputum were: Pseudomonas aeruginosa (33), Haemophilus influenzae (10), Moraxella catarrhalis (2), other Gram-negative bacilli (5), Streptococcus pneumoniae (6), Staphylococcus aureus (5), mycobacteria (3), and yeast (1). Clinical parameters in patients with positive isolation of P aeruginosa were compared with those without the organism in the sputum culture (non-P aeruginosa). In the P aeruginosa group, the FEV1/FVC ratio and sputum volume were lower (p < 0.005) and higher (p < 0.0001), respectively, than those of the non-P aeruginosa group. The FEV1/FVC ratio (< 60%) and sputum volume (grading > 5) were independently associated with a positive sputum isolation of P aeruginosa with odds ratios of 3.1 (confidence interval [CI] 1.2 to 8.4; p < 0.01) and 4.7 (CI 1.6 to 13.3; p < 0.001), respectively. CONCLUSIONS: P aeruginosa is the predominant respiratory pathogen isolated in the sputum of Chinese patients with steady-state bronchiectasis, and its isolation is associated with high sputum output (> or = 75th quartile) and moderately severe airflow obstruction (FEV1/FVC < 60%).

Adult↗

PubMed2004

Survival of patients with bronchiectasis after the first ICU stay for respiratory failure.

STUDY OBJECTIVES: Respiratory failure (RF) is a frequent cause of death among patients with bilateral bronchiectasis. An ICU admission is commonly required, and neither short-term or long-term outcomes have been studied. DESIGN: We performed a retrospective study over a 10-year period (January 1990 to March 2000). All patients with bilateral bronchiectasis admitted for the first time in the medical ICU for RF were reviewed. Patients with cystic fibrosis were excluded. MEASUREMENTS AND RESULTS: Forty-eight patients (mean age +/- SD, 63 +/- 11 years; mean simplified acute physiology score [SAPS] II, 32 +/- 12) of whom 25% received long-term oxygen therapy (LTOT) were identified. All the patients were treated with intensive medical care, associated with noninvasive ventilation in 13 patients (27%), and 26 patients (54%) required intubation. Nine patients (19%) died in the ICU. The 1-year mortality rate was 40%. Among the variables recorded at ICU admission, age > 65 years (p = 0.002), SAPS II score > 32 (p = 0.012), use of LTOT (p = 0.047), and intubation (p = 0.027) were associated with reduced survival in univariate analysis by Cox regression. Multivariate analysis by Cox proportional hazard model showed that age > 65 years (relative risk [RR], 2.70; 95% confidence interval [CI], 1.15 to 6.29) and use of LTOT (RR, 2.52; 95% CI, 1.15 to 5.54) were independently associated with reduced survival. CONCLUSIONS: We performed the first study providing information related to the impact of the first ICU stay for RF on long-term outcomes for patients with bilateral bronchiectasis. Age > 65 years and prior use of LTOT were associated with reduced survival.

Adult↗

PubMed2005

Quality-of-life determinants in patients with clinically stable bronchiectasis.

STUDY OBJECTIVE: To determine the most important variables influencing health-related quality of life (HRQL) in patients with clinically stable bronchiectasis (SB). DESIGN: Cross-sectional study. PATIENTS AND INTERVENTIONS: A total of 86 patients (mean age, 69.5 years; SD, 8.9 years; 64% male) with SB were included. Data were collected on general patient characteristics, symptoms, laboratory findings, the extent of bronchiectasis, functional variables, medication in acute or stable phases, and the number of exacerbations. All patients completed the St. George Respiratory Questionnaire (SGRQ). Univariate and multivariate analyses were performed to identify the variables significantly influencing HRQL in these patients. RESULTS: Different clinical parameters (sputum, dyspnea, cough, and wheezing), spirometric variables, and laboratory parameters (fibrinogen), as well as the extent of bronchiectasis, medication, and the number of exacerbations were significantly correlated to the total questionnaire score, although only dyspnea (r2 = 0.43, p < 0.0001), FEV1 (r2 = 0.33, p < 0.0001), and daily sputum production (r2 = 0.2, p < 0.004) were independently correlated to the total score, globally explaining 55% of the total score variability. Systemic steroid treatment of exacerbations (r2 = 0.17, p < 0.028) and the habitual presence of coughing (r2 = 0.22, p < 0.004) and wheezing (r2 = 0.16, p < 0.013) were in turn independently correlated to the activity and symptoms subscales, respectively. CONCLUSION: Dyspnea, FEV1, and sputum production are the strongest conditioning factors of HRQL in patients with clinically SB.

Adult↗

PubMed2006

Prospective analysis of cystic fibrosis transmembrane regulator mutations in adults with bronchiectasis or pulmonary nontuberculous mycobacterial infection.

BACKGROUND: Bronchiectasis and pulmonary infection with nontuberculous mycobacteria (NTM) may be associated with disease-causing mutations in the cystic fibrosis transmembrane regulator (CFTR). METHODS: Fifty adult patients at Stanford University Medical Center with a diagnosis of bronchiectasis and/or pulmonary NTM infection were prospectively characterized by sweat chloride measurement, comprehensive mutational analysis of CFTR, and sputum culture results. RESULTS: A de novo diagnosis of cystic fibrosis (CF) was established in 10 patients (20%). Patients with CF were more likely than those without CF to have mucus plugging seen on chest high-resolution CT, and women with a CF diagnosis were thinner, with a significantly lower mean body mass index than the non-CF subjects. Thirty CFTR mutations were identified in 24 patients (50% prevalence). Sweat chloride concentration was elevated > 60 mEq/dL (diagnostic of CF) in seven patients (14%), and from 40 to 60 mEq/dL in eight patients (16%). The frequency of CFTR mutations was elevated above that expected in the general population: heterozygous DeltaF508 (12% vs 3%), R75Q (14% vs 1%), and intron 8 5T (17% vs 5 to 10%). Other known CFTR mutations identified were V456A, G542X, R668C, I1027T, D1152, R1162L, W1282X, and L183I. Three novel CFTR mutations were identified: A394V, F650L, and C1344S. CONCLUSIONS: Mutations in CFTR that alter RNA splicing and/or functional chloride conductance are common in this population, and are likely to contribute to the susceptibility and pathogenesis of adult bronchiectasis and pulmonary NTM infection. Careful clinical evaluation for disease cause should be undertaken in this clinical context.

Adult↗

PubMed1975

Bronchiectasis and homozygous alpha1-antitrypsin deficiency.

A 34-year-old woman with homozygous a1-antitrypsin deficiency suffered from progressive, generalized cystic bronchiectasis. Although bronchiectasis was reported in the original monograph on the enzyme inhibitor deficiency, it has received minimal attention since then. Alpha1-antitrypsin levels should be measured in patients with severe bronchiectasis.

Adult↗

PubMed1977

Bronchiectasis and spontaneous pneumothorax in Marfan's syndrome.

A Chinese girl with Marfan's syndrome had spontaneous pneumothorax and bronchiectasis. Spontaneous pneumothorax is not an infrequent pulmonary manifestation of Marfan's syndrome, but bronchiectasis is rarely reported to occur. So far, it has not been reported that both bronchiectasis and spontaneous pneumothorax can occur in the same patient with Marfan's syndrome.

Adolescent↗