Bronchiectasis

Bronchiectasis

Overview

Bronchiectasis is a condition characterized by scarring and dilation of the bronchi (the airway leading to the lungs). This leads to chronic productive cough with mucopurulent sputum and other nonspecific respiratory symptoms. People also experience exacerbation (worsening) of symptoms sometimes. There are various genetic (like CF, primary ciliary dyskinesia) and environmental (smoking, fumes) causes identified. Other risk factors include pulmonary infections, airway obstruction, immunosuppression, some systemic and rheumatic disease, COPD, etc. The diagnosis is based on a chest CT or X-ray. Other examinations that can be useful are blood tests, sputum culture, bronchoscopy, and functional lung tests. There is no possible cure, but this condition can be managed with medications (like antibiotics and mucolytics) or special devices. If you have this condition, it is recommended to quit smoking, stay hydrated, and get vaccinated against pneumonia and flu. 

Prevalence & Risk factors

The prevalence of bronchiectasis ranges from 67 to 566 per 100 000 people in Europe and North America.

It increases with age and is higher in women. Two factors are required for the development of bronchiectasis: a local infection or inflammation and either impaired drainage, airway obstruction, or a defect in host defense. Sometimes the cause is unknown, then it’s called idiopathic bronchiectasis. There are several risk factors that have been identified:

  • Age
  • Sex (more common in women)
  • Airway obstruction (foreign body aspiration, compression with enlarged lymph nodes or some tumor, anatomic defects of the airways)
  • Defective host defenses (immunosuppression, hypogammaglobulinemia)
  • Cystic fibrosis 
  • Rheumatic and systemic diseases
  • Alpha-1-antitrypsin deficiency (an autosomal codominant genetic disease that causes damage to lungs and liver)
  • Primary ciliary dyskinesia (an autosomal recessive disorder characterized by chronic respiratory tract infections, abnormally positioned internal organs and infertility)
  • Pulmonary infections
  • Allergic bronchopulmonary aspergillosis (ABPA)
  • Smoking
  • COPD

   

Genetics

The most common inherited cause of bronchiectasis is cystic fibrosis - an autosomal recessive genetic disease that leads to damage to the lungs, GIT, kidneys, and other organs. There are other autosomal recessive and dominant disorders that can cause bronchiectasis and other genes can predispose to this condition. 

 

Signs & Symptoms

The typical symptom is a chronic productive cough with mucopurulent sputum that lasts for months to years. Rarely, the cough may be without sputum but with some blood (hemoptysis). Other less specific complaints include trouble breathing (dyspnea), wheezing, and pleuritic chest pain. People affected by bronchiectasis usually tend to have recurrent respiratory infections over years, requiring antibiotic therapy. Sometimes they can also experience acute exacerbation (worsening) of the symptoms. 

Diagnosis

The diagnosis is confirmed with an X-ray or a CT scan of the chest (showing dilated and thickened airways). Blood tests and sputum culture are done to identify the microbial agent causing inflammation. Your doctor might also perform a bronchoscopy for the visualization of the bronchi from inside or order lung function tests like spirometry.

Therapy

Bronchiectasis can’t be cured but it can be managed using medications, special devices, or a combination of those. The medications that can be used to improve the condition are antibiotics against infections and drugs that help to cough the mucus out. This can also be achieved using different airway clearance devices, like positive expiratory pressure (PEP) devices and percussive devices. Physical therapy like chest clapping helps with mucus excretion too. 

Prevention

There are some ways to prevent the development of bronchiectasis (unless there is an underlying genetic disease like CF). It is recommended to get vaccinated against pneumonia and flu, prevent any airway obstruction (especially in small children that might aspirate a foreign body), and avoid harmful fumes/gases. It’s also really important to quit smoking. Proper hydration can keep the mucus from getting too sticky and relieve the symptoms. 

Prognosis

With the proper management, the lifespan of people affected by bronchiectasis is normal. Some complications may arise, like COPD, pulmonary hemorrhages, or lung abscess.

Recommendations

  • If you experience any symptoms, please contact your doctor
  • Improve your health by quitting smoking and avoiding harmful fumes and gases
  • Get vaccinated against pneumonia and get the annual flu vaccination

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Recommendations

  • If you experience any symptoms, please contact your doctor
  • Improve your health by quitting smoking and avoiding harmful fumes and gases
  • Get vaccinated against pneumonia and get the annual flu vaccination

Sources

  1. Wei-jie Guan, Xiao-rong Han, David de la Rosa-Carrillo, Miguel Angel Martinez-Garcia, European Respiratory Journal 2019 53: 1802392; DOI: 10.1183/13993003.02392-2018
  2. Bronchiectasis; Causes, Symptoms, Treatment & Prevention https://my.clevelandclinic.org/health/diseases/21144-bronchiectasis
  3. Wei-jie Guan, Xiao-rong Han, David de la Rosa-Carrillo, Miguel Angel Martinez-Garcia, European Respiratory Journal 2019 53: 1802392; DOI: 10.1183/13993003.02392-2018
  4. Barker A.F., (2022), Clinical manifestations and diagnosis of bronchiectasis in adults, UptoDate, https://www-uptodate-com.ezproxy.is.cuni.cz/contents/clinical-manifestations-and-diagnosis-of-bronchiectasis-in-adults?search=bronchiectasis%20prevalence&source=search_result&selectedTitle=3~150&usage_type=default&display_rank=3

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