Defining severity in non-cystic fibrosis bronchiectasis

Abstract

Bronchiectasis is a common disease with important impacts on patient’s quality of life and on healthcare systems in terms of healthcare costs, hospitalisations and mortality. After decades of being regarded as an orphan disease there are an increasing numbers of treatments available, or in late-stage clinical trials. Assessment of disease severity is fundamental to clinical management. To date, however, there is no agreed definition of what constitutes mild, moderate or severe bronchiectasis. These terms are often applied to radiological appearances, but while important, computed tomography findings are not sufficiently precise to capture the complex impact of the disease. Studies are beginning to define the risk factors for mortality, hospital admissions, exacerbations and impaired quality of life in bronchiectasis, allowing us to propose new definitions of what constitutes severe bronchiectasis. An improved understanding of severity and prognosis in this disease will aid clinical decision making and the application of new therapies.

Keywords::

• bacteria
• bronchiectasis
• exacerbations
• infection
• mortality
• neutrophil
• prognosis
• severity

Financial & competing interests disclosure

JD Chalmers has received fees for speaking on advisory boards for GSK, AstraZeneca and Bayer HealthCare. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending or royalties.

No writing assistance was utilized in the production of this manuscript.

Key issues

• Understanding the severity of disease and prognosis is key to clinical decision-making in bronchiectasis.

• The key clinical end points are mortality, hospital admissions, exacerbations, health-related quality of life and symptoms.

• Initial studies suggest that the most consistent markers of future risk are increasing age, dyspnea, radiological severity of bronchiectasis, colonization with Pseudomonas aeruginosa, lower forced expiratory volume in 1 s, lower total lung capacity and a past history of exacerbations.

• Chronic obstructive pulmonary disease-related bronchiectasis appears to carry a particularly poor prognosis, while little data exist for other etiologies.

• Genetic and biomarkers studies have the potential to greatly improve risk stratification in bronchiectasis, but studies are at a very early stage.

• Multidimensional scoring systems for bronchiectasis have the potential to improve clinical management. A bronchiectasis severity index has been developed, which accurately stratifies risk of mortality, hospital admissions and exacerbations across five European bronchiectasis centers.

• Further data on prognosis and the clinical impact of prognostic tools are needed.