Bronchiectasis and asthma: Data from the European Bronchiectasis Registry (EMBARC).

BACKGROUND: Asthma is commonly reported in patients with a diagnosis of bronchiectasis. OBJECTIVE: The aim of this study was to evaluate whether patients with bronchiectasis and asthma (BE+A) had a different clinical phenotype and different outcomes compared with patients with bronchiectasis without concomitant asthma. METHODS: A prospective observational pan-European registry (European Multicentre Bronchiectasis Audit and Research Collaboration) enrolled patients across 28 countries. Adult patients with computed tomography-confirmed bronchiectasis were reviewed at baseline and annual follow-up visits using an electronic case report form. Asthma was diagnosed by the local investigator. Follow-up data were used to explore differences in exacerbation frequency between groups using a negative binomial regression model. Survival analysis used Cox proportional hazards regression. RESULTS: Of 16,963 patients with bronchiectasis included for analysis, 5,267 (31.0%) had investigator-reported asthma. Patients with BE+A were younger, were more likely to be female and never smokers, and had a higher body mass index than patients with bronchiectasis without asthma. BE+A was associated with a higher prevalence of rhinosinusitis and nasal polyps as well as eosinophilia and Aspergillus sensitization. BE+A had similar microbiology but significantly lower severity of disease using the bronchiectasis severity index. Patients with BE+A were at increased risk of exacerbation after adjustment for disease severity and multiple confounders. Inhaled corticosteroid (ICS) use was associated with reduced mortality in patients with BE+A (adjusted hazard ratio 0.78, 95% CI 0.63-0.95) and reduced risk of hospitalization (rate ratio 0.67, 95% CI 0.67-0.86) compared with control subjects without asthma and not receiving ICSs. CONCLUSIONS: BE+A was common and was associated with an increased risk of exacerbations and improved outcomes with ICS use. Unexpectedly we identified significantly lower mortality in patients with BE+A.

The Association between Bronchiectasis and Chronic Obstructive Pulmonary Disease: Data from the European Bronchiectasis Registry (EMBARC).

Rationale: COPD and bronchiectasis are commonly reported together. Studies report varying impacts of co-diagnosis on outcomes, which may be related to different definitions of disease used across studies. Objectives: To investigate the prevalence of chronic obstructive pulmonary disease (COPD) associated with bronchiectasis and its relationship with clinical outcomes. We further investigated the impact of implementing the standardized ROSE criteria (radiological bronchiectasis [R], obstruction [FEV1/FVC ratio <0.7; O], symptoms [S], and exposure [⩾10 pack-years of smoking; E]), an objective definition of the association of bronchiectasis with COPD. Methods: Analysis of the EMBARC (European Bronchiectasis Registry), a prospective observational study of patients with computed tomography-confirmed bronchiectasis from 28 countries. The ROSE criteria were used to objectively define the association of bronchiectasis with COPD. Key outcomes during a maximum of 5 years of follow-up were exacerbations, hospitalization, and mortality. Measurements and Main Results: A total of 16,730 patients with bronchiectasis were included; 4,336 had a clinician-assigned codiagnosis of COPD, and these patients had more exacerbations, worse quality of life, and higher severity scores. We observed marked overdiagnosis of COPD: 22.2% of patients with a diagnosis of COPD did not have airflow obstruction and 31.9% did not have a history of ⩾10 pack-years of smoking. Therefore, 2,157 patients (55.4%) met the ROSE criteria for COPD. Compared with patients without COPD, patients who met the ROSE criteria had increased risks of exacerbations and exacerbations resulting in hospitalization during follow-up (incidence rate ratio, 1.25; 95% confidence interval, 1.15-1.35; vs. incidence rate ratio, 1.69; 95% confidence interval, 1.51-1.90, respectively). Conclusions: The label of COPD is often applied to patients with bronchiectasis who do not have objective evidence of airflow obstruction or a smoking history. Patients with a clinical label of COPD have worse clinical outcomes.

ROSE: radiology, obstruction, symptoms and exposure - a Delphi consensus definition of the association of COPD and bronchiectasis by the EMBARC Airways Working Group.

INTRODUCTION: The coexistence of COPD and bronchiectasis seems to be common and associated with a worse prognosis than for either disease individually. However, no definition of this association exists to guide researchers and clinicians. METHODS: We conducted a Delphi survey involving expert pulmonologists and radiologists from Europe, Turkey and Israel in order to define the "COPD- [bronchiectasis] BE association".A panel of 16 experts from EMBARC selected 35 statements for the survey after reviewing scientific literature. Invited participants, selected on the basis of expertise, geographical and sex distribution, were asked to express agreement on the statements. Consensus was defined as a score of ≥6 points (scale 0 to 9) in ≥70% of answers across two scoring rounds. RESULTS: 102 (72.3%) out of 141 invited experts participated in the first round. Their response rate in the second round was 81%. The final consensus definition of "COPD-BE association" was: "The coexistence of (1) specific radiological findings (abnormal bronchial dilatation, airways visible within 1 cm of pleura and/or lack of tapering sign in ≥1 pulmonary segment and in >1 lobe) with (2) an obstructive pattern on spirometry ([forced expiratory volume in 1 s] FEV1/[forced vital capacity] FVC <0.7), (3) at least two characteristic symptoms (cough, expectoration, dyspnoea, fatigue, frequent infections) and (4) current or past exposure to smoke (≥10 pack-years) or other toxic agents (biomass, etc.)". These criteria form the acronym "ROSE" (Radiology, Obstruction, Symptoms, Exposure). CONCLUSIONS: The Delphi process formulated a European consensus definition of "COPD-BE association". We hope this definition will have broad applicability across clinical practice and research in the future.

Comorbidities and mortality risk factors for patients with bronchiectasis.

Introduction: Comorbidities in patients with bronchiectasis are common and have a significant impact on clinical outcomes, contributing to lower quality of life, lung function, and exacerbation frequency. At least 13 comorbidities have been associated with a higher risk of mortality in bronchiectasis patients. Nonetheless, the kind of relationship between bronchiectasis and comorbidities is heterogeneous and poorly understood.Areas covered: different biological mechanisms leading to bronchiectasis could have a role in the development of the associated comorbidities. Some comorbidities could have a causal relationship with bronchiectasis, possibly through a variable degree of systemic inflammation, such as in rheumatic disorders and bowel inflammatory diseases. Other comorbidities, such as COPD or asthma, could be associated through airway inflammation and there is an uncertain cause-effect relationship. Finally, shared risk factors could link different comorbidities to bronchiectasis such as in the case of cardiovascular diseases, where the known link between chronic systemic inflammation and pulmonary infection could play a significant role.Expert opinion: Although different tools have been developed to assess the role of comorbidities in bronchiectasis , we believe that the implementation of current strategies to manage them is absolutely necessary and could significantly improve long-term prognosis in patients with bronchiectasis.

Bronchiectasis phenotypes.

PURPOSE OF REVIEW: Bronchiectasis is a chronic respiratory disease with heterogeneous clinical manifestations and outcomes. Identifying clinical phenotypes could help in managing bronchiectasis patients and hopefully improve disease prognosis by adopting personalized treatment. We review the current literature on clinical phenotypes of bronchiectasis and try to highlight priorities for future research. RECENT FINDINGS: Different studies have tried to stratify bronchiectasis patients according to cause, microbiology, or associated conditions. In consideration of the huge heterogeneity of bronchiectasis different cluster analyses in bronchiectasis have also been performed. Unfortunately classification by cause is clinically meaningful only in a few conditions in which specific treatment is available or a specific management is recommended. This is the case, for instance, of allergic bronchopulmonary aspergillosis, immunodeficiencies or chronic airways comorbidities of bronchiectasis, such as chronic obstructive pulmonary disease and asthma. From a clinical perspective, the microbiological cluster stratification proposed by Aliberti et al. seems the most profitable one but still so a number of host-related factors have an unpredictable effect on clinical manifestations and outcomes of bronchiectasis. SUMMARY: Although numerous attempts to identify clinical phenotypes in bronchiectasis have been performed, currently there is need to further investigate the host-related factors (endotypes) that surely play a determinant role in disease severity.

Bronchiectasis in severe asthma: a distinct phenotype?

PURPOSE OF REVIEW: The presence of bronchiectasis has been described in about 30% of severe asthma patients. The coexistence of these two respiratory conditions poses new challenges from both clinical and research perspectives. We will review the available literature on this topic to discuss the existance of a specific clinical phenotype of asthma. RECENT FINDINGS: Despite the paucity of literature, the presence of bronchiectasis with severe asthma is associated with older age, chronic bronchial expectoration, rhinosinusitis, more frequent and severe exacerbations, neutrophilic airway inflammation and poor response to usual treatment. Conversely, asthma features are also described in bronchiectasis patients even in the absence of an appropriate diagnosis of asthma. In both cases, there is some evidence supporting the use of bronchodilators, macrolides and respiratory physiotherapy, while the use of inhaled corticosteroids and antibiotics is controversial. SUMMARY: Based on available evidence on the association between (severe) asthma and bronchiectasis, its pathophysiology, certain clinical aspects and prognosis are largely unclear. Although specific management appears to be required in most cases, in our opinion there is still insufficient evidence to consider it a distinct phenotype of severe asthma. Hopefully, future research will shed more light on this topic and define the best therapeutic approach.