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Bronchiectasis lungs iconBronchiectasis is a chronic and progressive disease often marked by unpredictable exacerbations 1

Bronchiectasis is a chronic lung disease usually marked by permanent, abnormal dilation and persistent inflammation of the airways. 1,2

Bronchiectasis affects approximately 450,000 people in the United States and more globally. 1,3 Although increasing in prevalence, there have long been unmet needs in bronchiectasis. 1,4

Healthy airway and bronchiectasis-inflamed airway comparison drawing

Etiologies commonly associated with bronchiectasis

Bronchiectasis has several known etiologies, including but not limited to 5,6:

  • Airway abnormalities1 toggle icon
    • Airway obstruction by tumor or foreign body
    • Congenital tracheobronchial abnormalities
      • Mounier-Kuhn syndrome
      • Williams-Campbell syndrome
      • Ehlers-Danlos syndrome
  • Allergic bronchopulmonary aspergillosis
  • Aspiration syndromes1 toggle icon
    • Vocal cord disease or dysfunction
    • Esophageal disease or dysmotility
  • Asthma
  • Autoimmune disorders and connective tissue diseases1 toggle icon
    • Rheumatoid arthritis
    • Sjögren’s syndrome
    • Scleroderma
    • Inflammatory bowel disease
  • Congenital disorders1 toggle icon
    • Cystic fibrosis
    • Primary ciliary dyskinesia
    • Alpha-1 antitrypsin deficiency
  • COPD
  • Immunodeficiencies1 toggle icon
    • Common variable immunodeficiency
    • Acquired immunodeficiency
    • HIV infection
    • Hematologic cancer
  • Post-infectious bronchiectasis1 toggle icon
    • After bacterial or viral pneumonia or tuberculosis

40%

Up to 40% of patients with bronchiectasis have no specific identifiable cause of disease determined. 5

The presentation of bronchiectasis often overlaps with other respiratory conditions 7,8

Bronchiectasis initially presents with nonspecific symptoms that mimic more common conditions such as COPD or asthma, which experts believe often delays diagnosis or leads to misdiagnoses. 5,8,9

Common symptoms associated with bronchiectasis 1,10,11:

  • Cough icon
    Chronic cough
  • Dyspnea icon
    Dyspnea
  • Sputum icon
    Daily sputum production
  • Fatigue eye icon
    Fatigue
  • Lungs icon
    Recurrent infections
  • Hemoptysis icon
    Hemoptysis

A definitive diagnosis of bronchiectasis requires both clinical criteria and radiological evidence. 1,9,11

  • Clinical suspicion includes presence of symptoms and history of exacerbations
  • Findings on imaging include dilated airways, lack of airway tapering, and airway visibility in the periphery

Exacerbations play a critical role in bronchiectasis disease progression, with serious consequences for patients 12

Patients with bronchiectasis often suffer from periods of worsening symptoms, or exacerbations, which contribute to the burden of disease and can affect patients’ quality of life. 12

Although exacerbations are considered separate from daily symptom burdens, many patients view them as a continuum of the disease and a part of daily life. 13 It’s important that patients are educated about the consequences of exacerbations and the appropriate actions to take, including when to seek medical help.

Identifying an exacerbation

There are no US guidelines that define a bronchiectasis exacerbation. However, a consensus definition from an expert committee defines an exacerbation as a patient experiencing the worsening of 3 or more of these symptoms over 48 hours, requiring a change in treatment 14:

  • Cough icon red
    Cough
  • Fatigue eye icon red
    Fatigue and/or malaise
  • Dyspnea icon red
    Breathlessness and/or exercise intolerance
  • Hemoptysis icon red
    Hemoptysis
  • Sputum icon red
    Sputum volume and/or consistency
  • Sputum purulence icon red
    Sputum purulence

Exacerbations contribute to an increased physical and mental burden for patients with bronchiectasis 1,12

Exacerbations can negatively affect patients and their families, and are associated with 1,12,13:

  • Significant anxiety
  • Lack of control
  • Decreased quality of life
  • Embarrassment
  • Work and social interruptions
Quotation mark graphicIt’s like waiting for a hurricane—there’s a constant level of fear and anxiety of the unknown. When will an exacerbation hit? How will my life be affected or change with this disease?Michelle, patient with bronchiectasis

2x

Compared to patients with no exacerbations, patients who have experienced an exacerbation have nearly double the risk for another, and the risk increases with subsequent exacerbations. 12

Most patients with bronchiectasis experience 1 or more exacerbations, resulting in greater disease burden over time 15

A retrospective study of US commercial claims and Medicare supplemental core databases included data from 14,798 bronchiectasis patients aged ≥18 years and found that a majority of bronchiectasis patients experienced 15:

  • 1 or more exacerbations at Years 1 and 2 of follow-up
  • Increased hospitalizations from Year 1 to Year 2

Pulmonary exacerbation* occurrences over time periods in patients with non-cystic fibrosis bronchiectasis (N=14,798) 15

Number of exacerbations:

0
1
2
≥3

1-year follow-up

  • n=4,818
    32.6
  • 3,098
    20.9
  • 2,083
    14.1
  • 4,799
    32.4
05101520253035404550

% of patients with
pulmonary exacerbations

2-year follow-up

  • n=3,475
    23.5
  • 2,554
    17.3
  • 1,935
    13.1
  • 6,834
    46.2
05101520253035404550

% of patients with
pulmonary exacerbations

*

Pulmonary exacerbation defined here as an inpatient claim with a non-cystic fibrosis bronchiectasis diagnosis or a healthcare interaction, followed by an antibiotic prescription within 7 days. 15

Source: adapted from Flume PA, et al. ERJ Open Res. 2023;00021-02023.

Copyright ©Flume PA, et al 2023 - This version is distributed under the terms of the Creative Commons Attribution Non-Commercial License 4.0.

Expert commentary: The impact of exacerbations on patients with bronchiectasis

Show transcript

Exacerbations are important for a lot of reasons. One is because patients feel poorly when they have them, and we would therefore like to try and prevent them, or treat them rapidly when they occur. So, we believe that exacerbations are part of what drives the ongoing inflammation and progression of bronchiectasis.

And a lot of that is why we’re trying to prevent these events, to try and prevent that progression of disease. But something we learned is, we tend to focus on the acute things, like the cough and the sputum production, and we measure things like missed days of work or school; and those are all important. But the other thing is these are systemic events. And even though their symptoms of cough or chest complaints may improve rapidly, we actually now have data that show that their physical functioning takes weeks before they recover. And that’s not unlike any other kind of lung infection. And that’s a factor I think we have to pay attention to.

You know, one of the reasons we want to prevent exacerbations is we know they’re associated with some bad outcomes, worsening quality of life, and, very importantly, disease progression, and even mortality. So we really need to focus on preventing these exacerbations for our patients.

When patients have bronchiectasis exacerbations, it may lead to, you know, frequent outpatient visits, frequent hospitalizations, if not treated appropriately, you know, worsening symptoms, worsening quality of life. Oftentimes, they’re trying to help themselves, right? So it leads to more time consumed at home with medical, you know, using certain medications or certain devices. It’s just very cumbersome for the patient when they’re faced with exacerbations. You want to, you know, try to optimize quality of life as best possible for these patients. You know, you want to prevent any sort of worsening lung function from exacerbations or any sort of, you know, worsening inflammation and worsening bronchiectasis, right? So I think it’s very important to identify these exacerbations and treat it appropriately early.

Bronchiectasis patients can experience serious long-term effects from exacerbations 12

Data from a study of 2,572 patients with bronchiectasis from 10 clinical centers across Europe and Israel showed that 12:

  • Patients with 2 or more exacerbations per year at baseline had a 60% increased risk of 5-year all-cause mortality
  • Patients with 3 or more exacerbations per year at baseline had an 86% increased risk of 5-year all-cause mortality

Survival by number of baseline exacerbations per year 12

Chart of survival rate by a number of baseline exacerbations per year

Source: Reprinted with permission of the American Thoracic Society. Copyright © 2023 American Thoracic Society. All rights reserved. The American Journal of Respiratory and Critical Care Medicine is an official journal of the American Thoracic Society.

Frequent bronchiectasis exacerbations are associated with disease progression, lower lung function, and worse outcomes. 12,16

Data have shown that patients with bronchiectasis experiencing multiple exacerbations have significantly worse symptom frequency and severity, more limitations to activities, and more disturbances in social and mental functioning compared with those with fewer exacerbations. 12,17

Preventing exacerbations can help make a positive impact on patients both physically and mentally. 1,12

Quotation mark graphicI’m always wondering what my tomorrows are going to be like, if they are going to be good or if they are going to be bad. I worry a lot and stress a lot. I have anxiety a lot because I never know.Erica, patient with bronchiectasis
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