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The New England Journal of Medicine Aug 2022
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Current Opinion in Infectious Diseases Apr 2018Bronchiectasis, once thought to be an orphan disease, is being diagnosed with increased frequency in the United States and around the world. The present review aims to... (Review)
Review
PURPOSE OF REVIEW
Bronchiectasis, once thought to be an orphan disease, is being diagnosed with increased frequency in the United States and around the world. The present review aims to provide an update on recent publications on the diagnosis and management of bronchiectasis.
RECENT FINDINGS
Two large bronchiectasis patient registries have published initial reports regarding demographics and other patient data in 2017. Updates on the microbiology, microbiome, and inflammation in patients with bronchiectasis are clarifying the complexities of airway infection in this disease. A consensus definition of 'exacerbation' in bronchiectasis has been agreed upon this year. Reports on novel treatments, including the repurposing of older therapies, have also been published in 2016-2017. A new European guideline for the management of adult bronchiectasis is also now available.
SUMMARY
Bronchiectasis, a resurgent disease, is now being better defined with a rapidly expanding portfolio of demographic, clinical, and therapeutic research and publications.
Topics: Age Factors; Bronchiectasis; Disease Management; Global Health; Humans; Prevalence
PubMed: 29489526
DOI: 10.1097/QCO.0000000000000445 -
Lancet (London, England) Sep 2018Bronchiectasis is characterised by pathological dilation of the airways. More specifically, the radiographic demonstration of airway enlargement is the common feature of... (Review)
Review
Bronchiectasis is characterised by pathological dilation of the airways. More specifically, the radiographic demonstration of airway enlargement is the common feature of a heterogeneous set of conditions and clinical presentations. No approved therapies exist for the condition other than for bronchiectasis caused by cystic fibrosis. The heterogeneity of bronchiectasis is a major challenge in clinical practice and the main reason for difficulty in achieving endpoints in clinical trials. Recent observations of the past 2 years have improved the understanding of physicians regarding bronchiectasis, and have indicated that it might be more effective to classify patients in a different way. Patients could be categorised according to a heterogeneous group of endotypes (defined by a distinct functional or pathobiological mechanism) or by clinical phenotypes (defined by relevant and common features of the disease). In doing so, more specific therapies needed to effectively treat patients might finally be developed. Here, we describe some of the recent advances in endotyping, genetics, and disease heterogeneity of bronchiectasis including observations related to the microbiome.
Topics: Autoimmune Diseases; Bronchiectasis; Cystic Fibrosis; Disease Progression; Genetic Predisposition to Disease; Humans; Immunocompetence; Microbiota; Mutation
PubMed: 30215383
DOI: 10.1016/S0140-6736(18)31767-7 -
European Respiratory Review : An... Dec 2019Bronchiectasis is a heterogeneous chronic disease. Heterogeneity characterises bronchiectasis not only in the stable state but also during exacerbations, despite... (Review)
Review
Bronchiectasis is a heterogeneous chronic disease. Heterogeneity characterises bronchiectasis not only in the stable state but also during exacerbations, despite evidence on clinical and biological aspects of bronchiectasis, exacerbations still remain poorly understood.Although the scientific community recognises that bacterial infection is a cornerstone in the development of bronchiectasis, there is a lack of data regarding other trigger factors for exacerbations. In addition, a huge amount of data suggest a primary role of neutrophils in the stable state and exacerbation of bronchiectasis, but the inflammatory reaction involves many other additional pathways. Cole's vicious cycle hypothesis illustrates how airway dysfunction, airway inflammation, infection and structural damage are linked. The introduction of the concept of a "vicious vortex" stresses the complexity of the relationships between the components of the cycle. In this model of disease, exacerbations work as a catalyst, accelerating the progression of disease. The roles of microbiology and inflammation need to be considered as closely linked and will need to be investigated in different ways to collect samples. Clinical and translational research is of paramount importance to achieve a better comprehension of the pathophysiology of bronchiectasis, microbiology and inflammation both in the stable state and during exacerbations.
Topics: Bronchiectasis; Disease Progression; Humans
PubMed: 31748420
DOI: 10.1183/16000617.0055-2019 -
Respiratory Medicine May 2020Bronchiectasis is a complex, chronic respiratory condition, characterized by frequent cough and exertional dyspnea due to a range of conditions that include inherited... (Review)
Review
Bronchiectasis is a complex, chronic respiratory condition, characterized by frequent cough and exertional dyspnea due to a range of conditions that include inherited mucociliary defects, inhalational airway injury, immunodeficiency states and prior respiratory infections. For years, bronchiectasis was classified as either being caused by cystic fibrosis or non-cystic fibrosis. Non-cystic fibrosis bronchiectasis, once considered an orphan disease, is more prevalent worldwide in part due to greater availability of chest computed tomographic imaging. Identification of the cause of non-cystic fibrosis bronchiectasis with the use of chest imaging, laboratory testing, and microbiologic assessment of airway secretions can lead to initiation of specific therapies aimed at slowing disease progression. Nonpharmacologic therapies such as airway clearance techniques and pulmonary rehabilitation improve patient symptoms. Inhaled corticosteroids should not be routinely prescribed unless concomitant asthma or COPD is present. Inhaled antibiotics prescribed to individuals with >3 exacerbations per year are well tolerated, reduce airway bacteria load and may reduce the frequency of exacerbations. Likewise, chronic macrolide therapy reduces the frequency of exacerbations. Medical therapies for cystic fibrosis bronchiectasis may not be effective in treatment of non-cystic fibrosis bronchiectasis.
Topics: Administration, Inhalation; Anti-Bacterial Agents; Bronchiectasis; Cystic Fibrosis; Disease Progression; Humans; Rare Diseases; Respiratory System; Respiratory Therapy; Respiratory Tract Infections; Tomography, X-Ray Computed
PubMed: 32250872
DOI: 10.1016/j.rmed.2020.105940 -
The Medical Journal of Australia Aug 2018Once neglected in research and underappreciated in practice, there is renewed interest in bronchiectasis unrelated to cystic fibrosis. Bronchiectasis is a chronic lung... (Review)
Review
Once neglected in research and underappreciated in practice, there is renewed interest in bronchiectasis unrelated to cystic fibrosis. Bronchiectasis is a chronic lung disease characterised by chronic cough, sputum production and recurrent pulmonary exacerbations. It is diagnosed radiologically on high resolution computed tomography chest scan by bronchial dilatation (wider than the accompanying artery). The causes of bronchiectasis are diverse and include previous respiratory tract infections, chronic obstructive pulmonary disease, asthma, immunodeficiency and connective tissue diseases. A large proportion of cases are idiopathic, reflecting our incomplete understanding of disease pathogenesis. Progress in the evidence base is reflected in the 2017 European management guidelines and the 2015 update to the Australian guidelines. Effective airway clearance remains the cornerstone of bronchiectasis management. This should be personalised and reviewed regularly by a respiratory physiotherapist. There is now robust evidence for the long term use of oral macrolide antibiotics in selected patients to reduce exacerbation frequency. The routine use of long term inhaled corticosteroids and/or long-acting bronchodilators should be avoided, unless concomitant chronic obstructive pulmonary disease or asthma exists. The evidence for nebulised agents including hypertonic saline, mannitol and antibiotics is evolving; however, access is challenging outside tertiary clinics, and nebulising equipment is required. Smokers should be supported to quit. All patients should receive influenza and pneumococcal vaccination. Patients with impaired exercise capacity should attend pulmonary rehabilitation. There is an important minority of patients for whom aetiology-specific treatment exists. The prevalence of bronchiectasis is increasing worldwide; however, the burden of disease within Australia is not well defined. To this end, the Australian Bronchiectasis Registry began recruitment in 2016 and is interoperable with the European and United States bronchiectasis registries to enable collaborative research. The recent addition of a bronchiectasis diagnosis-related group to the Australian Refined Diagnostic Related Group classification system will allow definition of the disease burden within the Australian hospital system.
Topics: Administration, Inhalation; Adult; Anti-Bacterial Agents; Australia; Bronchiectasis; Humans
PubMed: 30107772
DOI: 10.5694/mja17.01195 -
BMJ Clinical Evidence Feb 2015Bronchiectasis is usually a complication of previous lower respiratory infection and/or inflammation. It causes chronic cough, copious production of sputum (often... (Review)
Review
INTRODUCTION
Bronchiectasis is usually a complication of previous lower respiratory infection and/or inflammation. It causes chronic cough, copious production of sputum (often purulent), and recurrent infections, and may cause airway obstruction bearing some similarities with that seen in COPD. It may complicate respiratory conditions such as asthma or COPD. It can be associated with primary ciliary dyskinesia, primary immunodeficiencies, certain systemic diseases such as inflammatory bowel disease and rheumatoid arthritis, and foreign body inhalation. Bronchiectasis can be due to cystic fibrosis but this is excluded from this review.
METHODS AND OUTCOMES
We conducted a systematic review and aimed to answer the following clinical question: What are the effects of treatments in people with non-cystic fibrosis (non-CF) bronchiectasis? We searched: Medline, Embase, The Cochrane Library, and other important databases up to January 2014 (Clinical Evidence reviews are updated periodically; please check our website for the most up-to-date version of this review). We included harms alerts from relevant organisations such as the US Food and Drug Administration (FDA) and the UK Medicines and Healthcare products Regulatory Agency (MHRA). We performed a GRADE evaluation of the quality of evidence for interventions.
RESULTS
We found 23 studies that met our inclusion criteria.
CONCLUSIONS
In this systematic review we present information relating to the effectiveness and safety of the following interventions: airway clearance techniques, corticosteroids (inhaled), exercise or physical training, hyperosmolar agents (inhaled), mucolytics, prolonged-use antibiotics, and surgery.
Topics: Administration, Inhalation; Adrenal Cortex Hormones; Anti-Bacterial Agents; Bronchiectasis; Cough; Exercise; Expectorants; Humans; Treatment Outcome
PubMed: 25715965
DOI: No ID Found -
Respirology (Carlton, Vic.) Mar 2019This paper aims to provide physiological rationale for airway clearance, mucoactive therapy and pulmonary rehabilitation (PR) (or exercise interventions) in... (Review)
Review
This paper aims to provide physiological rationale for airway clearance, mucoactive therapy and pulmonary rehabilitation (PR) (or exercise interventions) in bronchiectasis. There is increasing emphasis on the role of airway clearance techniques (ACT) in the management of bronchiectasis. No single ACT has currently shown superior effect over another. Given the large range of different techniques available, consideration of the physiological effects underpinning a technique including expiratory flow, ventilation and oscillation, is essential to effectively personalize ACT. Key clinical trials of mucoactives in bronchiectasis are underway and will provide clarity on the role of these agents in the management of patients with bronchiectasis. Prescription of mucoactive therapies should be done in conjunction with ACT and therefore the mechanism of action of mucoactive drugs and their timing with ACT should be taken into consideration. PR and/or exercise training are recommended in all current bronchiectasis guidelines. There is a clear physiological rationale that muscle weakness and physical inactivity may play a role in disease progression as well as impacting health-related quality of life, frequency of pulmonary exacerbations and ability to mobilize sputum. However, there are residual unanswered questions surrounding the delivery and accessibility to PR. This review summarizes the physiological principles and supporting evidence for airway clearance, mucoactive medication and PR, which are key components in the management of bronchiectasis.
Topics: Breathing Exercises; Bronchiectasis; Disease Progression; Exercise Therapy; Expectorants; Humans; Mucociliary Clearance; Quality of Life; Respiratory Therapy; Sputum
PubMed: 30650472
DOI: 10.1111/resp.13459 -
Clinical Medicine (London, England) Nov 2021Bronchiectasis is a heterogeneous and increasingly prevalent chronic pulmonary disease that is associated with significant morbidity. In this review, we outline how... (Review)
Review
Bronchiectasis is a heterogeneous and increasingly prevalent chronic pulmonary disease that is associated with significant morbidity. In this review, we outline how patients with bronchiectasis may present clinically and describe an approach to its diagnosis, including how to identify an underlying aetiology. We discuss the important considerations when treating either acute exacerbations or stable disease and provide an overview of the role of long-term antimicrobials, airway clearance methods and other supportive management.
Topics: Anti-Bacterial Agents; Bronchiectasis; Humans
PubMed: 34862215
DOI: 10.7861/clinmed.2021-0651 -
The Lancet. Respiratory Medicine Sep 2018European Respiratory Society guidelines for the management of adult bronchiectasis highlight the paucity of treatment options available for patients with this disorder....
European Respiratory Society guidelines for the management of adult bronchiectasis highlight the paucity of treatment options available for patients with this disorder. No treatments have been licensed by regulatory agencies worldwide, and most therapies used in clinical practice are based on very little evidence. Development of new treatments is needed urgently. We did a systematic review of scientific literature and clinical trial registries to identify agents in early-to-late clinical development for bronchiectasis in adults. In this Review, we discuss the mechanisms and potential roles of emerging therapies, including drugs that target airway and systemic inflammation, mucociliary clearance, and epithelial dysfunction. To ensure these treatments achieve success in randomised clinical trials-and therefore reach patients-we propose a reassessment of the current approach to bronchiectasis. Although understanding of the pathophysiology of bronchiectasis is at an early stage, we argue that bronchiectasis is a heterogeneous disease with many different biological mechanisms that drive disease progression (endotypes), and therefore the so-called treatable traits approach used in asthma and chronic obstructive pulmonary disease could be applied to bronchiectasis, with future trials targeted at the specific disease subgroups most likely to benefit.
Topics: Adult; Bronchiectasis; Disease Progression; Humans; Mucociliary Clearance; Practice Guidelines as Topic; Precision Medicine; Randomized Controlled Trials as Topic
PubMed: 29478908
DOI: 10.1016/S2213-2600(18)30053-5