Bronchiectasis: Information & Bronchiectasis Specialists

Bronchiectasis develops primarily as a result of mucostasis (mucus retention) and recurrent infectious diseases. These lead to a cough with mucus production.

The three-layered sputum—consisting of foam, mucus, and pus—has a sweetish, bland odor. Mucus is usually coughed up in the morning or after a change in position.

In addition, as the disease progresses, 

• shortness of breath (in 75 percent of cases),
• coughing up blood (in approximately 50 percent of cases),
• watchglass nails (arched and rounded fingernails), and
• clubbing (thickening of the fingertips) due to respiratory failure, as well as
• cor pulmonale (a condition characterized by severe enlargement of the right ventricle, which is located upstream of the lungs)

may be observed.

Long-term inflammation of the bronchi often leads to the development of what is known as amyloidosis. This is the continuous deposition of the body’s own, but altered, proteins in the form of small protein filaments. These cannot be broken down and cause long-term damage to organ structures.

An exacerbation (worsening of symptoms) can occur approximately every 1.5 years. This is characterized by:

• Increased mucus production with coughing
• Increased shortness of breath
• Fever above 38 °
• Increasing wheezing during exhalation
• Decreased exercise tolerance
• Fatigue and a feeling of exhaustion
• Decreased lung function
• Radiological signs of infection

© Usman Zafar Paracha | AdobeStock

Bronchiectasis is often attributed to hereditary causes. Congenital defects that can lead to the development of bronchiectasis include

• cystic fibrosis or
• Kartagener syndrome.

In these conditions, bronchiectasis is a characteristic symptom. It develops

• as a result of increased mucus production in the airways (cystic fibrosis) or
• due to a congenital disorder of ciliary function and the bronchi’s self-cleaning mechanism (Kartagener syndrome).

In addition, bronchiectasis can develop post-infectiously as part of

• recurrent infectious diseases,
• advanced chronic obstructive pulmonary disease (COPD), and
• as a result of bronchial stenosis caused by inhaled foreign bodies or tumors

.

Infections caused by the following pathogens can lead to bronchiectasis:

• whooping cough
• Bacteria such as Pseudomonas aeruginosa and Haemophilus influenzae
• Viruses such as influenza, adenovirus, paramyxovirus, and HIV
• Tuberculosis
• Atypical mycobacteria (including the Mycobacterium avium complex in Lady Windermere syndrome)

The diseases listed above impair the bronchi’s self-cleaning mechanism. This leads to an increased accumulation of mucus in the bronchi. This mucus provides a breeding ground for inhaled pathogenic (disease-causing) germs and promotes their proliferation.

This leads to frequent lung infections, which, through their chronic inflammatory foci, attack the bronchial walls and destroy them over the long term. Ultimately, this results in the dilation of one or more bronchi (bronchiectasis).

Treatment approaches for mucus clearance and infection prevention are largely based on cystic fibrosis therapy. The treatment goals are:

• Treatment of the underlying disease
• Improvement of the bronchi’s self-cleaning mechanism
• Treatment and prevention of infection,
• Treatment of airway obstruction (narrowing)
• Treatment of chronic inflammation with oral and inhaled corticosteroids (steroid hormones)

Specific therapeutic measures also depend on the cause and severity of the bronchiectasis. If, for example, the condition is localized to one side, surgical intervention to remove the diseased tissue may be necessary.

The foundation of bronchiectasis treatment consists of respiratory and physical therapy measures to loosen and improve the clearance of bronchial secretions (secretion drainage).

This therapy, also known as bronchial hygiene, is primarily based on the following measures:

• morning positioning in the knee-elbow position
• Positioning and vibration massages
• Use of vibration devices such as the RC-Cornet or VRP1-Flutter
• Adequate fluid intake to thin the bronchial secretions
• Inhalation therapy to improve the flow properties of bronchial secretions and thus enable more effective clearance, using, for example, saline solution
• Breathing exercises and training of the respiratory muscles to mobilize bronchial secretions

In cases of airway narrowing, bronchospasm-relieving therapy with bronchodilators is recommended. These medications widen the bronchi. To enhance secretion clearance and promote ciliary activity,

• inhaled beta-2 adrenergic agonists, including
  • salbutamol,
  • formoterol,
  • indacaterol,
  • salmeterol
• anticholinergics, including ipratropium and tiotropium

are available.

Combination bronchodilators have a greater bronchodilatory effect than single-agent preparations because they potentiate each other’s effects.

In addition, antibiotic therapy should be administered to treat infections. Antibiotic therapy is typically characterized by higher doses and a longer treatment duration compared to uncomplicated infectious diseases.

Due to the increased susceptibility to infection, active immunization against influenza (viral flu) and pneumococci (bacteria) is recommended.