Overcoming Challenges in Non-Cystic Fibrosis Bronchiectasis Research

Research in non-cystic fibrosis bronchiectasis (NCFBE) is expanding, but challenges such as heterogeneity and limitations of single-center studies must be addressed through collaborative efforts to advance understanding and treatment strategies according to an article published in the American Journal of Respiratory and Critical Care Medicine.

Researchers have historically focused more on bronchiectasis due to cystic fibrosis. Still, there has been a noticeable increase in research and funding for NCFBE in the past decade. Despite these advancements, there are still limited therapeutic options and preventive strategies for patients with NCFBE, highlighting the need for further progress in research. NCFBE researchers are urged to learn from other diseases and adapt their approaches to develop new insights and therapies for better patient outcomes.

The lung responds to injury in limited ways, but the mechanisms of disease can vary greatly despite similar pathological and radiological findings. In interstitial lung disease, different disease processes result in lung fibrosis with unique responses to therapies. Similarly, in chronic obstructive pulmonary disease and asthma, attention has shifted towards identifying distinct phenotypes and treatable traits based on clinical features and therapy responsiveness. NCFBE is a complex syndrome with various immune response deficits and healing pathways, leading to diverse presentations and implications for treatment efficacy. It is crucial for clinicians to differentiate between different clinical phenotypes and avoid pooling them together for clearer therapeutic advances.

NCFBE researchers face the challenge of insidious onset and long evolution of the syndrome, making it difficult to determine causation in cross-sectional studies that often attract severe cases. To fully understand NCFBE, research must also include patients on the milder end of the symptom spectrum. The increased use of CT scans for other conditions has led to the detection of asymptomatic individuals meeting bronchiectasis criteria, raising questions about diagnosis and the normal aging process. Longitudinal studies are necessary to address these uncertainties and implications for NCFBE understanding.

Researching NCFBE is challenging due to limitations in patient numbers and confounding comorbidities, making collaboration necessary for success. Establishing and maintaining large collaborative groups for research in NCFBE is difficult financially, logistically, and politically. Moving forward, single-center groups will face limitations in producing definitive findings, while national registries play a key role in identifying and defining clear phenotypes in NCFBE. Longitudinal research will be essential in identifying key determinants of progression and potential therapies for changing the natural course of NCFBE.

“We now have a vast array of tools to categorize the host and microbial systems at play in NCFBE. Very large amounts of time and money will be wasted if we do not learn from other diseases, appreciate that the complexity of NCFBE cannot be addressed without multicenter paradigms, and refashion research agendas to deal with these issues,” said the researcher. 

“Those who collaborate best will undoubtedly have the best opportunity to be ultimately remembered as those who solved the key unknowns in NCFBE and improved care for our patients.”

Reference
Waterer G W. What changes in research focus do we need to advance care for patients with bronchiectasis? Am J Respir Crit Care Med. 2024;210(1). doi: 10.1164/rccm.202403-0498VP