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Respiratory Medicine May 2023Bronchiectasis (BE) is a chronic condition characterized by airway dilation as a consequence of a variety of pathogenic processes. It is often associated with persistent... (Review)
Review
Bronchiectasis (BE) is a chronic condition characterized by airway dilation as a consequence of a variety of pathogenic processes. It is often associated with persistent airway infection and an inflammatory response resulting in cough productive of purulent sputum, which has an adverse impact on quality of life. The prevalence of BE is increasing worldwide. Treatment guidelines exist for managing BE, but they are generally informed by a paucity of high-quality evidence. This review presents the findings of a scientific advisory board of experts held in the United States in November 2020. The main focus of the meeting was to identify unmet needs in BE and propose ways to identify research priorities for the management of BE, with a view to developing evidence-based treatment recommendations. Key issues identified include diagnosis, patient evaluation, promoting airway clearance and appropriate use of antimicrobials. Unmet needs include effective pharmacological agents to promote airway clearance and reduce inflammation, control of chronic infection, clinical endpoints to be used in the design of BE clinical trials, and more accurate classification of patients using phenotypes and endotypes to better guide treatment decisions and improve outcomes.
Topics: Humans; Quality of Life; Bronchiectasis; Cough; Chronic Disease
PubMed: 36931575
DOI: 10.1016/j.rmed.2023.107217 -
Pediatrics and Neonatology Jun 2020Pediatric non-cystic fibrosis (CF) bronchiectasis is characterized by endobronchial suppuration, airway neutrophilic inflammation and poor mucus clearance and is... (Review)
Review
Pediatric non-cystic fibrosis (CF) bronchiectasis is characterized by endobronchial suppuration, airway neutrophilic inflammation and poor mucus clearance and is associated with persistent productive cough due to recurrent airway infections. Most recommendations are based on expert opinion or extrapolated from CF practice. The present narrative review aims to address some issues on the management of children or adolescents with non CF-bronchiectasis that still require attention, and analyze what available literature offers to reply to open questions. We focused on the potential offered by technological advances on lung disease assessment through novel chest imaging techniques and new or old pulmonary function tests. We also summarized the main novelties in the disease prevention and treatment. Finally, a novel diagnostic algorithm is proposed, that might help physicians in the daily clinical decision-making process. Future directions for research on pediatric non-CF bronchiectasis should include larger study populations and longer prospective clinical trials, as well as new clinical and laboratory endpoints to determine the underlying mechanisms of lung disease progression and support the role of new and existing treatments.
Topics: Adolescent; Bronchiectasis; Child; Cystic Fibrosis; Humans; Prospective Studies
PubMed: 31672477
DOI: 10.1016/j.pedneo.2019.09.013 -
Clinical Medicine (London, England) Jan 2019
Review
Topics: Anti-Bacterial Agents; Bronchiectasis; Humans
PubMed: 30651248
DOI: 10.7861/clinmedicine.19-1-64 -
Seminars in Respiratory and Critical... Aug 2021Bronchiectasis is a heterogenous disease with multiple etiologies and associated comorbidities. As bronchiectasis is a complex disease, it is unsound to think of it as a...
Bronchiectasis is a heterogenous disease with multiple etiologies and associated comorbidities. As bronchiectasis is a complex disease, it is unsound to think of it as a single disease particularly when the differing etiologies are likely to be driving bronchiectasis through initial divergent molecular pathways, known as endotypes, that phenotypically present as the same disease due to protracted airway inflammation, but revealing potential differing underlying mechanisms that may have disparity of drug responses. Improved understanding of the cellular immune, inflammatory, and microbiological milieu associated with clinical and radiological features of bronchiectasis has resulted in the recognition of important endotypes and phenotypes that will allow for personalized treatments to improve quality of life and outcomes of patients with bronchiectasis. Here we discuss clinical and radiological phenotypes, as well as emerging molecular endotypes that are possible treatable traits in bronchiectasis.
Topics: Bronchiectasis; Humans; Phenotype; Quality of Life; Radiography
PubMed: 34261179
DOI: 10.1055/s-0041-1730894 -
Respirology (Carlton, Vic.) Feb 2019Bronchiectasis is a chronic lung disease associated with structurally abnormal bronchi, clinically manifested by a persistent wet/productive cough, airway infections and... (Review)
Review
Bronchiectasis is a chronic lung disease associated with structurally abnormal bronchi, clinically manifested by a persistent wet/productive cough, airway infections and recurrent exacerbations. Early identification and treatment of acute exacerbations is an integral part of monitoring and annual review, in both adults and children, to minimize further damage due to infection and inflammation. Common modalities used to monitor disease progression include clinical signs and symptoms, frequency of exacerbations and/or number of hospital admissions, lung function (forced expiratory volume in 1 s (FEV )% predicted), imaging (radiological severity of disease) and sputum microbiology (chronic infection with Pseudomonas aeruginosa). There is good evidence that these monitoring tools can be used to accurately assess severity of disease and predict prognosis in terms of mortality and future hospitalization. Other tools that are currently used in research settings such as health-related quality of life (QoL) questionnaires, magnetic resonance imaging and lung clearance index can be burdensome and require additional expertise or resource, which limits their use in clinical practice. Studies have demonstrated that cross-infection, especially with P. aeruginosa between patients with bronchiectasis is possible but infrequent. This should not limit participation of patients in group activities such as pulmonary rehabilitation, and simple infection control measures should be carried out to limit the risk of cross-transmission. A multidisciplinary approach to care which includes respiratory physicians, chest physiotherapists, nurse specialists and other allied health professionals are vital in providing holistic care. Patient education and personalized self-management plans are also important despite limited evidence it improves QoL or frequency of exacerbations.
Topics: Adult; Anti-Bacterial Agents; Bronchiectasis; Child; Diagnostic Techniques, Respiratory System; Disease Progression; Humans; Patient Care Team; Prognosis; Quality of Life; Respiratory Therapy; Respiratory Tract Infections; Self-Management
PubMed: 30500093
DOI: 10.1111/resp.13451 -
Respirology (Carlton, Vic.) Dec 2018Bronchiectasis is a chronic respiratory disorder characterized by persistent productive cough and recurrent chest infections secondary to permanent structural airway... (Review)
Review
Bronchiectasis is a chronic respiratory disorder characterized by persistent productive cough and recurrent chest infections secondary to permanent structural airway damage. The current treatment strategies for this debilitating disorder are limited to prompt antibiotic treatment of infective exacerbations and regular airway clearance techniques. Despite its high morbidity and associated mortality across all age groups, it has been a neglected area of research in respiratory medicine and there remain no licensed disease-modifying therapies. In this review, we have explored the numerous potential therapeutic targets to break the vicious cycle of infection and inflammation seen in these patients and the novel therapeutic agents that have been developed to target them. We have reviewed the role of novel anti-inflammatory agents designed to target the persistent neutrophilic inflammatory infiltrate seen in bronchiectatic airways, including neutrophil elastase inhibitors, CXCR2 (CXC chemokine receptor 2) antagonists, DPP-1 (dipeptidyl peptidase 1) inhibitors, PDE4 (phosphodiesterase 4) inhibitors and statins. Furthermore, we have explored novel targets to improve mucociliary clearance, namely ENaC (epithelial sodium channel) inhibitors, and discussed the potential of alternative antimicrobial strategies such as inhaled phages. Our review highlights the importance of a multi-faceted approach to bronchiectasis management, which aims not only to eradicate or suppress bronchial infection but also to break the cycle of persistent airway inflammation that results in progressive lung damage in these patients.
Topics: Adult; Anti-Bacterial Agents; Anti-Inflammatory Agents; Bronchiectasis; Child; Humans; Mucociliary Clearance; Patient Care Management
PubMed: 30242794
DOI: 10.1111/resp.13407 -
BMC Pulmonary Medicine May 2018Bronchiectasis is an incurable lung disease characterised by irreversible airway dilatation. It causes symptoms including chronic productive cough, dyspnoea, and... (Review)
Review
BACKGROUND
Bronchiectasis is an incurable lung disease characterised by irreversible airway dilatation. It causes symptoms including chronic productive cough, dyspnoea, and recurrent respiratory infections often requiring hospital admission. Fatigue and reductions in quality of life are also reported in bronchiectasis. Patients often require multi-modal treatments that can be burdensome, leading to issues with adherence. In this article we review the provision of, and requirement for, education and information in bronchiectasis.
DISCUSSION
To date, little research has been undertaken to improve self-management in bronchiectasis in comparison to other chronic conditions, such as COPD, for which there has been a wealth of recent developments. Qualitative work has begun to establish that information deficit is one of the potential barriers to self-management, and that patients feel having credible information is fundamental when learning to live with and manage bronchiectasis. Emerging research offers some insights into ways of improving treatment adherence and approaches to self-management education; highlighting ways of addressing the specific unmet information needs of patients and their families who are living with bronchiectasis.
CONCLUSIONS
We propose non-pharmacological recommendations to optimise patient self-management and symptom recognition; with the aim of facilitating measurable improvements in health outcomes for patients with bronchiectasis.
Topics: Access to Information; Bronchiectasis; Chronic Disease; Health Education; Humans; Patient Education as Topic; Self-Management
PubMed: 29788946
DOI: 10.1186/s12890-018-0633-5 -
The European Respiratory Journal Nov 2022Bronchiectasis is being diagnosed increasingly in children and adolescents. Recurrent respiratory exacerbations are common in children and adolescents with this chronic...
Bronchiectasis is being diagnosed increasingly in children and adolescents. Recurrent respiratory exacerbations are common in children and adolescents with this chronic pulmonary disorder. Respiratory exacerbations are associated with an impaired quality of life, poorer long-term clinical outcomes, and substantial costs to the family and health systems. The 2021 European Respiratory Society (ERS) clinical practice guideline for the management of children and adolescents with bronchiectasis provided a definition of acute respiratory exacerbations for clinical use but to date there is no comparable universal definition for clinical research. Given the importance of exacerbations in the field, this ERS Task Force sought to obtain robust definitions of respiratory exacerbations for clinical research. The panel was a multidisciplinary team of specialists in paediatric and adult respiratory medicine, infectious disease, physiotherapy, primary care, nursing, radiology, methodology, patient advocacy, and parents of children and adolescents with bronchiectasis. We used a standardised process that included a systematic literature review, parent survey, and a Delphi approach involving 299 physicians (54 countries) caring for children and adolescents with bronchiectasis. Consensus was obtained for all four statements drafted by the panel as the disagreement rate was very low (range 3.6-7.2%). The panel unanimously endorsed the four consensus definitions for 1a) non-severe exacerbation and 1b) severe exacerbation as an outcome measure, 2) non-severe exacerbation for studies initiating treatment, and 3) resolution of a non-severe exacerbation for clinical trials involving children and adolescents with bronchiectasis. This ERS Task Force proposes using these internationally derived, consensus-based definitions of respiratory exacerbations for future clinical paediatric bronchiectasis research.
Topics: Adult; Adolescent; Child; Humans; Anti-Bacterial Agents; Quality of Life; Bronchiectasis; Respiratory System; Outcome Assessment, Health Care
PubMed: 35728974
DOI: 10.1183/13993003.00300-2022 -
Respirology (Carlton, Vic.) Nov 2019Bronchiectasis, conventionally defined as irreversible dilatation of the bronchial tree, is generally suspected on a clinical basis and confirmed by means of chest... (Review)
Review
Bronchiectasis, conventionally defined as irreversible dilatation of the bronchial tree, is generally suspected on a clinical basis and confirmed by means of chest high-resolution computed tomography. Clinical manifestations, including chronic productive cough and endobronchial suppuration with persistent chest infection and inflammation, may deeply affect quality of life, both in children/adolescents and adults. Despite many cases being idiopathic or post-infectious, a number of specific aetiologies have been traditionally associated with bronchiectasis, such as cystic fibrosis (CF), primary ciliary dyskinesia or immunodeficiencies. Nevertheless, bronchiectasis may also develop in patients with bronchial asthma; chronic obstructive pulmonary disease; and, less commonly, rheumatological disorders and inflammatory bowel diseases. Available literature on the development of bronchiectasis in these conditions and on its management is limited, particularly in children. However, bronchiectasis may complicate the clinical course of the underlying condition at any age, and appropriate management requires an integration of multiple skills in a team of complementary experts to provide the most appropriate care to affected children and adolescents. The present review aims at summarizing the current knowledge and available evidence on the management of bronchiectasis in the other conditions mentioned and focuses on the new therapeutic strategies that are emerging as promising tools for improving patients' quality of life.
Topics: Adult; Asthma; Bronchiectasis; Child; Comorbidity; Humans; Inflammatory Bowel Diseases; Patient Care Management; Pulmonary Disease, Chronic Obstructive; Rheumatic Diseases
PubMed: 31222879
DOI: 10.1111/resp.13615 -
Therapeutic Advances in Respiratory... 2022Bronchiectasis is associated with an increased incidence of atherosclerotic cardiovascular disease (ASCaVD) and atherosclerotic cerebrovascular disease (ASCeVD). Its...
BACKGROUND
Bronchiectasis is associated with an increased incidence of atherosclerotic cardiovascular disease (ASCaVD) and atherosclerotic cerebrovascular disease (ASCeVD). Its effect on associated mortality is unclear.
OBJECTIVES
This study investigated the effects of bronchiectasis exacerbation prior to ASCaVD or ASCeVD events on mortality in patients with bronchiectasis using a large population-based database.
METHODS
A retrospective cohort of patients with bronchiectasis who experienced ASCaVD ( = 1066) or ASCeVD ( = 825) was studied for the first time using a nationwide population-based database (National Health Insurance Service-National Sample Cohort, Korea, 2002-2015). We classified each cohort according to the presence of moderate bronchiectasis exacerbation within 1 year before the ASCaVD or ASCeVD event. We evaluated 90-day, 1-year, and all-cause mortalities risk.
RESULTS
Within 1 year before the index ASCaVD or ASCeVD event, 149 (13.9%) and 112 (13.6%) patients with bronchiectasis experienced moderate exacerbation(s), respectively. Mild exacerbations did not different in frequency between the survivors and nonsurvivors. In both cohorts, more nonsurvivors experienced moderate exacerbations than survivors. The odds ratios of 90-day and 1-year mortalities and hazard ratios of all-cause mortalities on experiencing moderate exacerbations were 2.27 [95% confidence interval (CI) = 1.26-4.10], 3.30 (95% CI = 2.03-5.38), and 1.78 (95% CI = 1.35-2.34) in the bronchiectasis-ASCaVD cohort and 1.73 (95% CI = 0.94-3.19), 1.79 (95% CI = 1.07-3.00), and 1.47 (95% CI = 1.10-1.95), in the bronchiectasis-ASCeVD cohort.
CONCLUSION
Hospitalization or emergency room visit for bronchiectasis exacerbation within 1 year before ASCaVD or ASCeVD is associated with an increased ASCaVD- or ASCeVD-associated mortality.
Topics: Humans; Retrospective Studies; Bronchiectasis; Lung; Hospitalization; Proportional Hazards Models; Disease Progression
PubMed: 36533883
DOI: 10.1177/17534666221144206