Seminars in respiratory and critical care medicine
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Semin Respir Crit Care Med · Aug 2021
Bronchiectasis Exacerbations: Definitions, Causes, and Acute Management.
Pulmonary exacerbations (PExs) are events in the course of bronchiectasis which are defined as an increase in disease symptoms lasting a period of a few days. It is established that the tendency toward having PEx is stable throughout the course of the disease. Certain conditions were found to be associated with an increased risk of developing a PEx. ⋯ Other acute changes such as air pollution, viral infection, and changes in bacterial metabolic activity have also been implicated as causes of a PEx. Despite these gaps in our knowledge of the biology of PEx, antimicrobial therapy directed against the identified pathogens in sputum is currently the recommended therapeutic strategy. Various long-term therapies, including antimicrobial and anti-inflammatory strategies, have been proven effective in reducing the frequency of PEx, leading to a recommendation for the use of these strategies in people with frequent PEx.
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A significant proportion of bronchiectasis patients are chronically infected by potentially pathogenic microorganisms which may lead to frequent exacerbations and worse clinical outcomes. Current bronchiectasis guidelines recommend long-term inhaled antibiotics and/or oral macrolides as a part of patient management. In recent years, an increasing amount of evidence assessing the impact of these treatments on patient outcomes has been collected. ⋯ In this regard, recent post hoc analyses of inhaled antibiotics trials in bronchiectasis patients have shown that sputum bacterial load may be a key biomarker to predict treatment response in these patients. Oral macrolides, on the other hand, have proven to reduce exacerbation frequency and improve quality of life, but potential drug-related adverse effects and the increase in bacterial resistance are relevant. This review aims to summarize current important evidence for long-term antibiotic treatment in bronchiectasis patients.
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Semin Respir Crit Care Med · Aug 2021
ReviewThe Deteriorating Patient: Therapies Including Lung Transplantation.
In this review paper, we discuss the characteristics that define severe bronchiectasis and which may lead to deterioration of noncystic fibrosis bronchiectasis. These characteristics were used to establish the current severity scores: bronchiectasis severity index (BSI), FACED, and E-FACED (exacerbation frequency, forced expiratory volume in 1 second, age, colonization, extension and dyspnea score). They can be used to predict mortality, exacerbation rate, hospital admission, and quality of life. ⋯ Finally, in patients who are difficult to treat despite maximum medical treatment, eligibility for surgery (when disease is localized), should be considered. In case of end-stage disease, the evaluation for lung transplantation should be performed. Noninvasive ventilation can serve as a bridge to lung transplantation in patients with respiratory failure.
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Semin Respir Crit Care Med · Aug 2021
Common Variable Immunodeficiency and Other Immunodeficiency Syndromes in Bronchiectasis.
Immunodeficiency represents a vast number of diseases and syndromes. Both primary and secondary forms of immunodeficiency are important contributors to the development of bronchiectasis. Primary immune deficiencies, in particular, are increasingly identified and defined as contributors. ⋯ Graft versus host disease, malignancy, and human immunodeficiency virus are three classic examples discussed in this article. An awareness of the potential for these disease settings to lead to bronchiectasis is necessary to optimize patient care. With understanding and mindfulness toward the intricate relationship between bronchiectasis and immunodeficiency, there is an opportunity to elucidate pathophysiologic underpinnings between these two syndromes.
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The respiratory system is constantly exposed to external pathogens but has different and effective defense systems. The pathophysiology of bronchiectasis affects the defense system considerably in that alterations occur in the airway that reduce its effectiveness in mucociliary clearance and the greater presence of mucins leads to the accumulation of more adherent and viscous mucus. ⋯ The properties of mucus can be modified to improve its mobilization through the use of a mucoactive agent. Despite the increase in the number and quality of studies, the evidence for these treatments remains scarce, although their application is recommended in all guidelines.