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Australian Journal of General Practice Dec 2022Bronchiectasis is a common chronic lung disease that is prevalent in the Australian community and is a significant disease burden in the Australian Aboriginal community....
BACKGROUND
Bronchiectasis is a common chronic lung disease that is prevalent in the Australian community and is a significant disease burden in the Australian Aboriginal community. It significantly affects quality of life and increases healthcare costs; however, it is poorly reported and documented.
OBJECTIVE
The aim of this article is to provide a comprehensive summary of the important features to recognise in bronchiectasis and an updated guide to the management of bronchiectasis.
DISCUSSION
Bronchiectasis is an obstructive lung disease that is often associated with various underlying conditions that can occur as a result of infectious insult or impaired host defence. The main features of bronchiectasis are dilated airways with increased sputum production, recurrent exacerbations and impaired quality of life. Management is aimed at investigating and managing secondary causes with a focus on sputum clearance, reduction in exacerbations and improvement of quality of life.
Topics: Humans; Quality of Life; Australia; Bronchiectasis; Cost of Illness
PubMed: 36451317
DOI: 10.31128/AJGP-08-22-6520 -
Pulmonology 2023Bronchiectasis is a highly complex entity that can be very challenging to investigate and manage. Patients are diverse in their aetiology, symptoms, risk of... (Meta-Analysis)
Meta-Analysis Review
Bronchiectasis is a highly complex entity that can be very challenging to investigate and manage. Patients are diverse in their aetiology, symptoms, risk of complications and outcomes. "Endotypes"- subtypes of disease with distinct biological mechanisms, has been proposed as a means of better managing bronchiectasis. This review discusses the emerging field of endotyping in bronchiectasis. We searched PubMed and Google Scholar for randomized controlled trials (RCT), observational studies, systematic reviews and meta-analysis published from inception until October 2022, using the terms: "bronchiectasis", "endotypes", "biomarkers", "microbiome" and "inflammation". Exclusion criteria included commentaries and non-English language articles as well as case reports. Duplicate articles between databases were initially identified and appropriately excluded. Studies identified suggest that it is possible to classify bronchiectasis patients into multiple endotypes deriving from their co-morbidities or underlying causes to complex infective or inflammatory endotypes. Specific biomarkers closely related to a particular endotype might be used to determine response to treatment and prognosis. The most clearly defined examples of endotypes in bronchiectasis are the underlying causes such as immunodeficiency or allergic bronchopulmonary aspergillosis where the underlying causes are clearly related to a specific treatment. The heterogeneity of bronchiectasis extends, however, far beyond aetiology and it is now possible to identify subtypes of disease based on inflammatory mechanisms such airway neutrophil extracellular traps and eosinophilia. In future biomarkers of host response and infection, including the microbiome may be useful to guide treatments and to increase the success of randomized trials. Advances in the understanding the inflammatory pathways, microbiome, and genetics in bronchiectasis are key to move towards a personalized medicine in bronchiectasis.
Topics: Humans; Precision Medicine; Bronchiectasis; Biomarkers; Inflammation; Comorbidity
PubMed: 37030997
DOI: 10.1016/j.pulmoe.2023.03.004 -
American Journal of Respiratory and... Mar 2014There are no risk stratification tools for morbidity and mortality in bronchiectasis. Identifying patients at risk of exacerbations, hospital admissions, and mortality...
RATIONALE
There are no risk stratification tools for morbidity and mortality in bronchiectasis. Identifying patients at risk of exacerbations, hospital admissions, and mortality is vital for future research.
OBJECTIVES
This study describes the derivation and validation of the Bronchiectasis Severity Index (BSI).
METHODS
Derivation of the BSI used data from a prospective cohort study (Edinburgh, UK, 2008-2012) enrolling 608 patients. Cox proportional hazard regression was used to identify independent predictors of mortality and hospitalization over 4-year follow-up. The score was validated in independent cohorts from Dundee, UK (n = 218); Leuven, Belgium (n = 253); Monza, Italy (n = 105); and Newcastle, UK (n = 126).
MEASUREMENTS AND MAIN RESULTS
Independent predictors of future hospitalization were prior hospital admissions, Medical Research Council dyspnea score greater than or equal to 4, FEV1 < 30% predicted, Pseudomonas aeruginosa colonization, colonization with other pathogenic organisms, and three or more lobes involved on high-resolution computed tomography. Independent predictors of mortality were older age, low FEV1, lower body mass index, prior hospitalization, and three or more exacerbations in the year before the study. The derived BSI predicted mortality and hospitalization: area under the receiver operator characteristic curve (AUC) 0.80 (95% confidence interval, 0.74-0.86) for mortality and AUC 0.88 (95% confidence interval, 0.84-0.91) for hospitalization, respectively. There was a clear difference in exacerbation frequency and quality of life using the St. George's Respiratory Questionnaire between patients classified as low, intermediate, and high risk by the score (P < 0.0001 for all comparisons). In the validation cohorts, the AUC for mortality ranged from 0.81 to 0.84 and for hospitalization from 0.80 to 0.88.
CONCLUSIONS
The BSI is a useful clinical predictive tool that identifies patients at risk of future mortality, hospitalization, and exacerbations across healthcare systems.
Topics: Adult; Aged; Aged, 80 and over; Bronchiectasis; Decision Support Techniques; Disease Progression; Exercise Test; Female; Follow-Up Studies; Hospitalization; Humans; Male; Middle Aged; Prognosis; Proportional Hazards Models; Prospective Studies; Respiratory Function Tests; Risk Assessment; Risk Factors; Severity of Illness Index; Surveys and Questionnaires
PubMed: 24328736
DOI: 10.1164/rccm.201309-1575OC -
The European Respiratory Journal Nov 2022Bronchiectasis can result from infectious, genetic, immunological and allergic causes. 60-80% of cases are idiopathic, but a well-recognised genetic cause is the motile... (Observational Study)
Observational Study
BACKGROUND
Bronchiectasis can result from infectious, genetic, immunological and allergic causes. 60-80% of cases are idiopathic, but a well-recognised genetic cause is the motile ciliopathy, primary ciliary dyskinesia (PCD). Diagnosis of PCD has management implications including addressing comorbidities, implementing genetic and fertility counselling and future access to PCD-specific treatments. Diagnostic testing can be complex; however, PCD genetic testing is moving rapidly from research into clinical diagnostics and would confirm the cause of bronchiectasis.
METHODS
This observational study used genetic data from severe bronchiectasis patients recruited to the UK 100,000 Genomes Project and patients referred for gene panel testing within a tertiary respiratory hospital. Patients referred for genetic testing due to clinical suspicion of PCD were excluded from both analyses. Data were accessed from the British Thoracic Society audit, to investigate whether motile ciliopathies are underdiagnosed in people with bronchiectasis in the UK.
RESULTS
Pathogenic or likely pathogenic variants were identified in motile ciliopathy genes in 17 (12%) out of 142 individuals by whole-genome sequencing. Similarly, in a single centre with access to pathological diagnostic facilities, 5-10% of patients received a PCD diagnosis by gene panel, often linked to normal/inconclusive nasal nitric oxide and cilia functional test results. In 4898 audited patients with bronchiectasis, <2% were tested for PCD and <1% received genetic testing.
CONCLUSIONS
PCD is underdiagnosed as a cause of bronchiectasis. Increased uptake of genetic testing may help to identify bronchiectasis due to motile ciliopathies and ensure appropriate management.
Topics: Humans; Mutation; Bronchiectasis; Cilia; Ciliary Motility Disorders; Ciliopathies; Kartagener Syndrome
PubMed: 35728977
DOI: 10.1183/13993003.00176-2022 -
The Cochrane Database of Systematic... Sep 2017People with bronchiectasis experience chronic cough and sputum production and require the prescription of airway clearance techniques (ACTs). A common type of ACT... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
People with bronchiectasis experience chronic cough and sputum production and require the prescription of airway clearance techniques (ACTs). A common type of ACT prescribed is positive expiratory pressure (PEP) therapy. A previous review has suggested that ACTs including PEP therapy are beneficial compared to no treatment in people with bronchiectasis. However, the efficacy of PEP therapy in a stable clinical state or during an acute exacerbation compared to other ACTs in bronchiectasis is unknown.
OBJECTIVES
The primary aim of this review was to determine the effects of PEP therapy compared with other ACTs on health-related quality of life (HRQOL), rate of acute exacerbations, and incidence of hospitalisation in individuals with stable or an acute exacerbation of bronchiectasis.Secondary aims included determining the effects of PEP therapy upon physiological outcomes and clinical signs and symptoms compared with other ACTs in individuals with stable or an acute exacerbation of bronchiectasis.
SEARCH METHODS
We searched the Cochrane Airways Group Specialised Register of Trials, PEDro and clinical trials registries from inception to February 2017 and we handsearched relevant journals.
SELECTION CRITERIA
Randomised controlled parallel and cross-over trials that compared PEP therapy versus other ACTs in participants with bronchiectasis.
DATA COLLECTION AND ANALYSIS
We used standard methodological procedures as outlined by Cochrane.
MAIN RESULTS
Nine studies involving 213 participants met the inclusion criteria, of which seven were cross-over in design. All studies included adults with bronchiectasis, with eight including participants in a stable clinical state and one including participants experiencing an acute exacerbation. Eight studies used oscillatory PEP therapy, using either a Flutter or Acapella device and one study used Minimal PEP therapy. The comparison intervention differed between studies. The methodological quality of studies was poor, with cross-over studies including suboptimal or no washout period, and a lack of blinding of participants, therapists or personnel for outcome measure assessment in most studies. Clinical heterogeneity between studies limited meta-analysis.Daily use of oscillatory PEP therapy for four weeks was associated with improved general health according to the Short-Form 36 questionnaire compared to the active cycle of breathing technique (ACBT). When applied for three sessions over one week, minimal PEP therapy resulted in similar improvement in cough-related quality of life as autogenic drainage (AD) and L'expiration Lente Totale Glotte Ouverte en Decubitus Lateral (ELTGOL). Oscillatory PEP therapy twice daily for four weeks had similar effects on disease-specific HRQOL (MD -0.09, 95% CI -0.37 to 0.19; low-quality evidence). Data were not available to determine the incidence of hospitalisation or rate of exacerbation in clinically stable participants.Two studies of a single session comparison of oscillatory PEP therapy and gravity-assisted drainage (GAD) with ACBT had contrasting findings. One study found a similar sputum weight produced with both techniques (SMD 0.54g (-0.38 to 1.46; 20 participants); the other found greater sputum expectoration with GAD and ACBT (SMD 5.6 g (95% CI 2.91 to 8.29: 36 participants). There was no difference in sputum weight yielded between oscillatory PEP therapy and ACBT with GAD when applied daily for four weeks or during an acute exacerbation. Although a single session of oscillatory PEP therapy was associated with less sputum compared to AD (median difference 3.1 g (95% CI 1.5 to 4.8 g; one study, 31 participants), no difference between oscillatory PEP therapy and seated ACBT was evident. PEP therapy had a similar effect on dynamic and static measures of lung volumes and gas exchange as all other ACTs. A single session of oscillatory PEP therapy (Flutter) generated a similar level of fatigue as ACBT with GAD, but greater fatigue was noted with oscillatory PEP therapy compared to ACBT alone. The degree of breathlessness experienced with PEP therapy did not differ from other techniques. Among studies exploring adverse events, only one study reported nausea with use of oscillatory PEP therapy.
AUTHORS' CONCLUSIONS
PEP therapy appears to have similar effects on HRQOL, symptoms of breathlessness, sputum expectoration, and lung volumes compared to other ACTs when prescribed within a stable clinical state or during an acute exacerbation. The number of studies and the overall quality of the evidence were both low. In view of the chronic nature of bronchiectasis, additional information is needed to establish the long-term clinical effects of PEP therapy over other ACTs for outcomes that are important to people with bronchiectasis and on clinical parameters which impact on disease progression and patient morbidity in individuals with stable bronchiectasis. In addition, the role of PEP therapy during an acute exacerbation requires further exploration. This information is necessary to provide further guidance for prescription of PEP therapy for people with bronchiectasis.
Topics: Aged; Bronchiectasis; Cough; Disease Progression; Hospitalization; Humans; Middle Aged; Positive-Pressure Respiration; Quality of Life; Randomized Controlled Trials as Topic; Respiratory Therapy; Sputum
PubMed: 28952156
DOI: 10.1002/14651858.CD011699.pub2 -
European Respiratory Review : An... Sep 2019Bronchiectasis is increasing in prevalence worldwide, yet current treatments available are limited to those alleviating symptoms and reducing exacerbations. The... (Review)
Review
Bronchiectasis is increasing in prevalence worldwide, yet current treatments available are limited to those alleviating symptoms and reducing exacerbations. The pathogenesis of the disease and the inflammatory, infective and molecular drivers of disease progression are not fully understood, making the development of novel treatments challenging. Understanding the role bacteria play in disease progression has been enhanced by the use of next-generation sequencing techniques such as 16S rRNA sequencing. The microbiome has not been extensively studied in bronchiectasis, but existing data show lung bacterial communities dominated by , and , while exhibiting intraindividual stability and large interindividual variability. and -dominated microbiomes have been shown to be linked to severe disease and frequent exacerbations. Studies completed to date are limited in size and do not fully represent all clinically observed disease subtypes. Further research is required to understand the microbiomes role in bronchiectasis disease progression. This review discusses recent developments and future perspectives on the lung microbiome in bronchiectasis.
Topics: Animals; Anti-Bacterial Agents; Bronchiectasis; Disease Progression; Haemophilus; Host-Pathogen Interactions; Humans; Lung; Microbiota; Pseudomonas; Risk Factors; Streptococcus
PubMed: 31484665
DOI: 10.1183/16000617.0048-2019 -
Respiratory Medicine Jul 2016Bronchiectasis is a condition of increasing incidence and prevalence around the world. Many different diseases have been associated with bronchiectasis, and their... (Review)
Review
Bronchiectasis is a condition of increasing incidence and prevalence around the world. Many different diseases have been associated with bronchiectasis, and their treatment can differ widely. Recent guidelines have helped to approach aetiological diagnosis but it is still a complex process. Identifying the cause of the bronchiectasis may determine a change in the treatment of a large group of subjects. That is one of the main reasons why the aetiological diagnosis is crucial in the proper management of bronchiectasis patients. Postinfectious bronchiectasis is the most frequent entity among different studies, but a high percentage of cases still remain without a clear aetiology. Bronchiectasis related to allergic bronchopulmonary aspergillosis (ABPA), immunodeficiencies with antibody production deficiency, primary ciliary dyskinesia, cystic fibrosis and alpha-1-antitrypsin deficiency, among others, require a specific management that may improve quality of life and prognosis in a large group of individuals. Therefore, the aim of this article is to review the main bronchiectasis related diseases and to simplify the aetiological diagnosis, in order to improve the management of bronchiectasis patients, especially in those where a specific treatment is available.
Topics: Adult; Aged; Aspergillosis, Allergic Bronchopulmonary; Bronchiectasis; Connective Tissue Diseases; Cystic Fibrosis; Female; Humans; Immunologic Deficiency Syndromes; Incidence; Inflammatory Bowel Diseases; Kartagener Syndrome; Male; Middle Aged; Prevalence; Quality of Life; alpha 1-Antitrypsin Deficiency
PubMed: 27296824
DOI: 10.1016/j.rmed.2016.05.014 -
Respirology (Carlton, Vic.) Feb 2019Bronchiectasis has received increased attention recently, including an emphasis on preventing infective exacerbations that are associated with disease progression and... (Review)
Review
Bronchiectasis has received increased attention recently, including an emphasis on preventing infective exacerbations that are associated with disease progression and lung function decline. While there are several bacteria and viruses associated with bronchiectasis, licensed vaccines are only currently available for Streptococcus pneumoniae, Haemophilus influenzae (H. influenzae protein D as a conjugate in a pneumococcal vaccine), Mycobacterium tuberculosis, Bordetella pertussis and influenza virus. The evidence for the efficacy and effectiveness of these vaccines in both preventing and managing bronchiectasis in children and adults is limited with the focus of most research being on other chronic lung disorders, such as chronic obstructive pulmonary diseases, asthma and cystic fibrosis. We review the existing evidence for these vaccines in bronchiectasis and highlight the existing gaps in knowledge. High-quality experimental and non-experimental studies using current state-of-the-art microbiological methods and validated, standardised case definitions are needed across the depth and breadth of the vaccine development pathway.
Topics: Adult; Bronchiectasis; Child; Disease Progression; Humans; Needs Assessment; Respiratory Tract Infections; Vaccination; Vaccines
PubMed: 30477047
DOI: 10.1111/resp.13446 -
Deutsches Arzteblatt International Dec 2011Radiologically evident bronchiectasis is seen in 30% to 50% of patients with advanced chronic obstructive pulmonary disease (COPD). As COPD is now becoming more common... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Radiologically evident bronchiectasis is seen in 30% to 50% of patients with advanced chronic obstructive pulmonary disease (COPD). As COPD is now becoming more common around the world, bronchiectasis is as well.
METHODS
We review pertinent articles published before May 2011 that were retrieved by a selective PubMed search.
RESULTS
The principles of treatment of bronchiectasis in patients who do not have cystic fibrosis ("non-CF bronchiectasis") are derived from the treatment of other diseases: secretolytic and anti-infectious treatment are given as in cystic fibrosis, while anti-obstructive treatment is given as in COPD. The few randomized trials of treatment for non-CF bronchiectasis that have been completed to date do not permit the formulation of any evidence-based recommendations. Many potential treatments are now under evaluation. Hypertonic saline is often used because of its demonstrated benefit in CF, even though no benefit has yet been shown for non-CF bronchiectasis. Phase II trials of inhaled mannitol have yielded promising results, leading to phase III trials that are now underway. There may be a future role for inhaled antibiotics, particularly in patients colonized with Gram-negative pathogens. Inhaled tobramycin and colistin are well established in clinical practice, though not approved for non-CF bronchiectasis; clinical trials of aztreonam, ciprofloxacin, and gentamicin are ongoing. Macrolides seem to bring an additional benefit, though the studies that documented this involved only small numbers of patients. Long-term treatment with inhaled antibiotics and/or macrolides is indicated only if a benefit is seen within three months of the start of treatment (less sputum, no exacerbations).
CONCLUSION
A national registry of patients with bronchiectasis should be established to help us gain better knowledge of its prognostic factors and treatment options.
Topics: Administration, Inhalation; Anti-Bacterial Agents; Bronchiectasis; Humans; Macrolides; Mannitol; Prevalence; Saline Solution, Hypertonic; Treatment Outcome
PubMed: 22211147
DOI: 10.3238/arztebl.2011.0809 -
Respiratory Research Dec 2017The role of neutrophil elastase (NE) is poorly understood in bronchiectasis because of the lack of preclinical data and so most of the assumptions made about NE... (Review)
Review
The role of neutrophil elastase (NE) is poorly understood in bronchiectasis because of the lack of preclinical data and so most of the assumptions made about NE inhibitor potential benefit is based on data from CF. In this context, NE seems to be a predictor of long-term clinical outcomes and a possible target of treatment. In order to better evaluate the role of NE in bronchiectasis, a systematic search of scientific evidence was performed.Two investigators independently performed the search on PubMed and included studies published up to May 15, 2017 according to predefined criteria. A final pool of 31 studies was included in the systematic review, with a total of 2679 patients. For each paper data of interest were extracted and reported in table.In this review sputum NE has proved useful as an inflammatory marker both in stable state bronchiectasis and during exacerbations and local or systemic antibiotic treatment. NE has also been associated with risk of exacerbation, time to next exacerbation and all-cause mortality. This study reviews also the role of NE as a specific target of treatment in bronchiectasis. Inhibition of NE is at a very early stage and future interventional studies should evaluate safety and efficacy for new molecules and formulations.
Topics: Biomarkers; Bronchiectasis; Cross-Sectional Studies; Humans; Leukocyte Elastase; Sputum
PubMed: 29258516
DOI: 10.1186/s12931-017-0691-x