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Editorial |

Bronchiectasis: An Orphan Finds a Home FREE TO VIEW

Pamela J. McShane, MD, FCCP
Author and Funding Information

FINANCIAL/NONFINANCIAL DISCLOSURES: None declared.

Section of Pulmonary and Critical Care Medicine, Department of Medicine, University of Chicago Medical Center, Chicago, IL

CORRESPONDENCE TO: Pamela J. McShane, MD, FCCP, Section of Pulmonary and Critical Care Medicine, Department of Medicine, 5841 S Maryland Ave, MC 6076, Chicago, IL 60637


Copyright 2017, American College of Chest Physicians. All Rights Reserved.


Chest. 2017;151(5):953-954. doi:10.1016/j.chest.2016.12.026
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In this issue of Chest, Aksamit and colleagues of the Bronchiectasis Research Registry (BRR) describe their national bronchiectasis cohort. The BRR is a large, U.S. consolidated database that exists under the umbrella of the COPD Foundation. It was created in 2007 at the recommendation of experts from the COPD Foundation in collaboration with the National Heart, Lung, and Blood Institute to better characterize non-CF patients with bronchiectasis and to serve as a reservoir from which to recruit patients for clinical trials and research. In 2011, a nontuberculous mycobacteria (NTM) component was added to the Registry in recognition of the link between the two diseases. At present, 13 clinical centers supply the centralized database with patient data. In essence, the BRR represents a unified, cohesive, national effort to take ownership of non-CF bronchiectasis, which has classically been referred to as an “orphan disease.”

FOR RELATED ARTICLE SEE PAGE 982

To our knowledge, the report by Aksamit and colleagues is the first official look at the national bronchiectasis cohort. It includes 1,826 analyzed patients who were enrolled between 2008 and 2014 and categorized by NTM status. Consistent with prior, smaller studies, the patients in the current study are predominantly female, white, nonsmokers with a mean age of 64 ± 14 years and mild-to-moderate obstruction on spirometry. Approximately one-half of the patient cohort was found to be covered by either Medicare or Medicaid. This is particularly noteworthy given current uncertainty regarding health-care reform and in light of the fact that prior research has shown that bronchiectasis is a more expensive disease to treat than many other chronic illnesses.

The present study reveals clinical and microbiologic associations that may be of pathophysiologic significance: gastroesophageal reflux disease was more frequently identified in those with NTM, but asthma, primary ciliary dyskinesia, and immunodeficiency were more common in patients without NTM. Sixty-three percent of patients had coexistent NTM and 33% grew Pseudomonas species on cultures, which was statistically significantly more common among NTM patients without NTM as compared with patients with NTM.

The study also provides insight into the current approach to the treatment of patients with bronchiectasis. For example, nonpharmacologic bronchial hygiene (chest percussion, or use of a flutter or PEP [positive expiratory pressure] valve) was performed in only 50% of patients without NTM; this despite data demonstrating improvements in health-related quality of life, sputum volume, and exercise capacity from airway clearance modalities in patients with bronchiectasis., In addition, inhaled steroids were used in 39% of the entire cohort despite a lack of supporting evidence for these drugs in patients with bronchiectasis. Thus, by revealing treatment styles, the BRR provides a basis from which to educate physicians.

As the BRR grows in number of clinical sites and patients, it is reasonable to expect that future research goals in bronchiectasis will be achieved. These goals include the following: obtaining a deeper understanding of bronchiectasis phenotypes and their determinants; a better understanding of the role that bacteria and the “microbiome” play in bronchiectasis; identification of additional therapies for the disease while improving our current approaches to treatment; and most important, identification of the mechanisms underlying the formation of bronchiectasis.

How can these goals be accelerated by the BRR? Inclusion of more minorities, and of more racial and ethnic groups, is crucial to enhance study development and opportunities for learning. At the national level, the investigators and clinicians who make up the BRR are collaborative, true advocates for patients with bronchiectasis, and supportive of new members of their community. At the international level, the BRR community has united with its European counterpart, the European Multicenter Bronchiectasis Audit and Research Collaboration (EMBARC), which houses a pan-European multicenter bronchiectasis database with objectives analogous to those of the U.S. BRR. As evidence of their alliance, the 1st World Bronchiectasis Conference, with a theme of “joining forces for a breakthrough in bronchiectasis,” took place in Hannover, Germany this past July. During the 3-day scientific program, more than 250 bronchiectasis researchers and clinicians from across Europe and America worked to establish cohesive and parallel goals, set consensus definitions, and prioritize unmet needs.

Finally, while it may be mere semantics to some, by entitling the article “Adult Bronchiectasis Patients: A First Look at the United States Bronchiectasis Research Registry,” the authors not only specify patients with bronchiectasis who do not have cystic fibrosis, they also reclaim the name “bronchiectasis” (heretofore “non-CF bronchiectasis).” This is a distinction that seemed odd given that bronchiectasis, a disease with a prevalence as high as 30:1,000, has been traditionally qualified by its rarer counterpart, cystic fibrosis, a disease that affects 1:2,500 by highest estimates. From now forward, patients with bronchiectasis have a home and a name.

References

Aksamit T.R. .O’Donnell A.E. .Barker A. . Bronchiectasis Research Registry Consortiumet al Adult patients with bronchiectasis: a first look at the US Bronchiectasis Research Registry. Chest. 2017;151:982-992 [PubMed]journal
 
Barker A. .Bardana E. Jr.. Bronchiectasis: update on an orphan disease. Am Rev Respir Dis. 1988;137:969-978 [PubMed]journal. [CrossRef] [PubMed]
 
Weycker D. .Edelsberg J. .Oster G. .Tino G. . Prevalence and economic burden of bronchiectasis. Clin Pulm Med. 2005;12:205-209 [PubMed]journal. [CrossRef]
 
Murray M. .Pentland J. .Hill A. . A randomised crossover trial of chest physiotherapy in non-cystic fibrosis bronchiectasis. Eur Respir J. 2009;34:1086-1092 [PubMed]journal. [CrossRef] [PubMed]
 
Lee A.L. .Burge A.T. .Holland A.E. . Airway clearance techniques for bronchiectasis. Cochrane Database Syst Rev. 2015;11:CD008351- [PubMed]journal
 
Aliberti S. .Masefield S. .Polverino E. . EMBARC Study Groupet al Research priorities in bronchiectasis: a consensus statement from the EMBARC Clinical Research Collaboration. Eur Respir J. 2016;48:632-647 [PubMed]journal. [CrossRef] [PubMed]
 
Seitz A. .Olivier K. .Adjemian J. .Holland S. .Prevot R. . Trends in bronchiectasis among Medicare beneficiaries in the United States, 2000 to 2007. Chest. 2012;142:432-439 [PubMed]journal. [CrossRef] [PubMed]
 
Cystic Fibrosis Foundation. Patient Registry 2014 Annual Data Report.https://www.cff.org/2014_CFF_Annual_Data_Report_to_the_Center_Directors.pdf/. Accessed January 18, 2017.
 

Figures

Tables

References

Aksamit T.R. .O’Donnell A.E. .Barker A. . Bronchiectasis Research Registry Consortiumet al Adult patients with bronchiectasis: a first look at the US Bronchiectasis Research Registry. Chest. 2017;151:982-992 [PubMed]journal
 
Barker A. .Bardana E. Jr.. Bronchiectasis: update on an orphan disease. Am Rev Respir Dis. 1988;137:969-978 [PubMed]journal. [CrossRef] [PubMed]
 
Weycker D. .Edelsberg J. .Oster G. .Tino G. . Prevalence and economic burden of bronchiectasis. Clin Pulm Med. 2005;12:205-209 [PubMed]journal. [CrossRef]
 
Murray M. .Pentland J. .Hill A. . A randomised crossover trial of chest physiotherapy in non-cystic fibrosis bronchiectasis. Eur Respir J. 2009;34:1086-1092 [PubMed]journal. [CrossRef] [PubMed]
 
Lee A.L. .Burge A.T. .Holland A.E. . Airway clearance techniques for bronchiectasis. Cochrane Database Syst Rev. 2015;11:CD008351- [PubMed]journal
 
Aliberti S. .Masefield S. .Polverino E. . EMBARC Study Groupet al Research priorities in bronchiectasis: a consensus statement from the EMBARC Clinical Research Collaboration. Eur Respir J. 2016;48:632-647 [PubMed]journal. [CrossRef] [PubMed]
 
Seitz A. .Olivier K. .Adjemian J. .Holland S. .Prevot R. . Trends in bronchiectasis among Medicare beneficiaries in the United States, 2000 to 2007. Chest. 2012;142:432-439 [PubMed]journal. [CrossRef] [PubMed]
 
Cystic Fibrosis Foundation. Patient Registry 2014 Annual Data Report.https://www.cff.org/2014_CFF_Annual_Data_Report_to_the_Center_Directors.pdf/. Accessed January 18, 2017.
 
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