Seminars in respiratory and critical care medicine
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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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Bronchiectasis is a complex, heterogeneous disorder defined by both a radiological abnormality of permanent bronchial dilatation and a clinical syndrome. There are multiple underlying causes including severe infections, mycobacterial disease, autoimmune conditions, hypersensitivity disorders, and genetic conditions. The pathophysiology of disease is understood in terms of interdependent concepts of chronic infection, inflammation, impaired mucociliary clearance, and structural lung damage. ⋯ The contribution of chronic inflammation, infection, and mucus obstruction leads to progressive structural lung damage. The heterogeneity of the disease is the most challenging aspect of management. An understanding of the pathophysiology of disease and their biomarkers can help to guide personalized medicine approaches utilizing the concept of "treatable traits."
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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.
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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.