Bronchiectasis: Advances in Diagnosis and Treatment

James D. Chalmers, MBChB, PhD, FRCPE, FERS

“Bronchiectasis is one of the most neglected diseases in respiratory medicine. Currently there is no clear definition or classification of the condition and very little is known about its true prevalence or its impact on length and quality of life. There are no specifically licensed therapies and few specialist clinical services. Significant research is needed in order to improve the quality of care of patients with this condition.”

–European Respiratory Society “White Book,” 2013

Bronchiectasis is a devastating disease, reducing both length and quality of life in its sufferers and placing a significant burden of the healthcare system. The quote above from the ERS White Book describes the long-term neglect of this disease, born from a belief that bronchiectasis would disappear once widespread sanitation, vaccination, and antibiotic treatment reduced the impact of communicable diseases, particularly in high-income countries. This belief was mistaken, and the prevalence of bronchiectasis has continued to grow throughout the world.

It is remarkable, however, to look back on the above quote from 2013 and consider how far the field of bronchiectasis has progressed.

Addressing this challenge has required increased awareness, intensive research, and the development of global treatment guidelines and specialist services. Most within respiratory medicine would regard the progress made over the past 10 years in bronchiectasis as remarkable. In contrast to the quote above, we now have clear definitions and classifications of disease. Multiple studies describe the prevalence, while since 2013 quality-of-life tools, multidimensional severity assessment tools, and large-scale registries such as EMBARC have informed on the impact of the disease on quality and length of life. There are still no licensed therapies and there remain many unanswered questions, but we have a far more robust evidence base for treatments such as airway clearance, macrolides, and inhaled antibiotics than we had in 2013. There have never been so many randomized trials in bronchiectasis with promising nonantibiotic approaches to the disease now in phase 3 trials. The field has been transformed inside a decade.

This issue in Seminars in Respiratory and Critical Care Medicine presents the state of the art on the topic of bronchiectasis. Key issues are discussed including our emerging understanding of the pathophysiology of disease which is leading to the development of new therapies.

The heterogeneity of bronchiectasis has long been considered its greatest challenge. Since 2013, several key concepts have emerged which are helping to breakdown the heterogeneity of bronchiectasis. The first is the concept of treatable traits. Treatable traits are defined as therapeutic targets which are identified through a detectable phenotype or endotype. To detect a phenotype or endotype a laboratory test, biomarker or important clinical characteristic must be identified. Whether we prefer the term “treatable traits,” phenotyping, endotyping, or personalized and precision medicine, these concepts are now central to how we investigate and treat bronchiectasis.

It is for this reason that we have chosen world leading experts in their field to review the most important treatable traits in bronchiectasis. Starting with a discussion of the pathophysiology, the importance of diagnostic testing, and the clinical and radiological phenotypes and endotypes of bronchiectasis, authors in this series outline how best to evaluate patients across the disease spectrum. We then present articles on immunodeficiency, primary ciliary dyskinesia, Aspergillus disease, and nontuberculous mycobacterial infection. Why these topics? Because these are the most common and most important treatable underlying causes of bronchiectasis. Identification of these diseases leads to different management strategies that can improve outcome making them “etiological” treatable traits.

The second part of the series reviews the latest in treatment including the crucial role of airway clearance, antibiotic therapy, the importance of treating Pseudomonas aeruginosa and considerations during exacerbations, and the management of the deteriorating patient. Taking together, these articles provide a comprehensive overview of the randomized trials, clinical experience, and guidelines that inform our current practice as well as considering directions for the future.

Patients with bronchiectasis can take encouragement from the great progress that has been made in recent years. Bronchiectasis is no longer neglected, and the brilliant clinicians and scientists who have contributed articles to this series will continue working toward treatments that can effectively alleviate and prevent the disease.

I very much hope you enjoy reading this issue of Seminars on bronchiectasis.

Publikationsverlauf

Artikel online veröffentlicht:
14. Juli 2021