Bronchiectasis: Information & bronchiectasis specialists

Leading Medicine Guide Editors
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Leading Medicine Guide Editors

Bronchiectasis are irreversible dilatations in the walls of the bronchi and their branches, the bronchioles. There are saccular, cylindrical and varicose bronchiectasis. All three forms develop as a result of genetic or acquired destruction of the muscle or elastic connective tissue in the affected bronchial structures.

Here you will find further information as well as selected specialists and centers for bronchiectasis.

ICD codes for this diseases: J47

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Article overview

Bronchiectasis can be

  • unilateral or bilateral,
  • diffuse (in different areas) or
  • locally limited

occur. Secretions accumulate in the dilated bronchi due to the impaired self-cleaning mechanism of the bronchi. This can be colonized by germs. Infections with inflammatory processes occur repeatedly. The disease becomes chronic and leads to progressive epithelial destruction.

The disease is diagnosed in 6 to 10 per 100,000 people every year.

Symptoms of bronchiectasis

Bronchiectasis is mainly caused by mucostasis (retention of mucus) and recurrent infections. These lead to coughing with mucus formation.

The three-layered sputum - consisting of foam, mucus and pus - smells sweetly bland. Mucus is usually expelled in the morning or after changes in position.

In addition, as the disease progresses

  • Shortness of breath (in 75 percent of cases),
  • Coughing up blood (in approx. 50 percent of cases),
  • watch glass nails (curved and rounded fingernails) and
  • drumstick fingers (thickening of the ends of the fingers) as a result of respiratory insufficiency and
  • cor pulmonale (pulmonary heart or severe dilatation of the right ventricle upstream of the lungs)

can be observed.

If the bronchi have been inflamed for many years, so-called amyloidosis often develops. This is a continuous deposition of the body's own but altered proteins in the form of small protein threads. They cannot be broken down and damage the organ structures in the long term.

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

  • Increase in phlegm with coughing
  • Increase in breathlessness
  • Fever above 38°
  • Increasing whistling exhalation noise
  • Decrease in resilience
  • Tiredness and feeling of exhaustion
  • Decrease in lung function
  • Radiological signs of infection
Die Lunge und ihre Anatomie
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    How do bronchiectasis develop?

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

    • Cystic fib rosis (cystic fibrosis) or
    • Kartagener's syndrome.

    Bronchiectasis is a characteristic symptom of these diseases. They form

    • as a result of increased secretion formation in the airways (cystic fibrosis) or
    • due to a congenital disorder of cilia activity and the self-cleaning mechanism of the bronchi (Kartagener's syndrome).

    In addition, bronchiectasis can develop post-infectiously in the context of

    manifest themselves.

    Infections with the following pathogens can lead to bronchiectasis:

    • Whooping cough
    • Bacteria such as Pseudomonas aeruginosa and Haemophilus influenzae
    • Viruses such as influenza, adenovirus, paramyxovirus, HIV
    • tuberculosis
    • Atypical mycobacteria (e.g. Mycobacterium avium complex in Lady Windermere syndrome)

    The diseases mentioned cause an impairment of the self-cleaning mechanism of the bronchi. This leads to an increased accumulation of mucus in the bronchial tubes. This provides a breeding ground for inhaled pathogenic (disease-causing) germs and promotes their multiplication.

    This leads to frequent lung infections, which attack and destroy the bronchial walls in the long term due to their chronic inflammation. This ultimately leads to the expansion of individual or several bronchi (bronchiectasis).

    Therapeutic measures for bronchiectasis

    The therapy concepts for clearing secretions and preventing infection are largely based on cystic fibrosis therapy. The treatment goals are

    • Therapy of the underlying disease
    • Improvement of the self-cleaning mechanism of the bronchi
    • Infection treatment and prophylaxis,
    • Treatment of airway obstruction (narrowing)
    • Treatment of chronic inflammation with oral and inhaled corticosteroids (steroid hormones)

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

    Bronchiectasis therapy is based on respiratory and physiotherapeutic measures to loosen and improve the removal of bronchial secretions (secretion drainage).

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

    • morning positioning in the knee-elbow position
    • Positioning and vibration massages
    • use of vibration aids such as RC-Cornet, VRP1-Flutter
    • Adequate fluid intake to liquefy the bronchial secretions
    • Inhalation therapy to improve the flow properties of bronchial secretions and thus more effective cleansing with, for example, saline solution
    • Respiratory gymnastics and training of the respiratory muscles to mobilize bronchial secretions

    If the airways are constricted, bronchospasmolytic therapy with bronchodilators is useful. These drugs dilate the bronchi. To increase secretion movement and promote ciliary activity, the following are available

    • Beta-2 sympathomimetics to be taken by inhalation, including
      • Salbutamol,
      • formoterol,
      • indacaterol,
      • salmeterol
    • anticholinergics, including ipratropium and tiotropium

    are available.

    Combined bronchodilators have a widening effect compared to single preparations, as they reinforce each other.

    Antibiotic therapy should also be carried out to treat the infections. Antibiotic therapy is usually characterized by higher doses and a longer treatment period compared to uncomplicated infectious diseases.

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

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