Author
Presentation by Dr. Urte Sommerwerck, summarized by Sabine Habicht | As published in Alpha1 Journal 2/2025.
„If there’s mucus, something might be wrong…“, Marion Wilkens announced in her welcoming remarks the lecture by Dr. Urte Sommerwerck on „Alpha1 and Bronchiectasis“, thus formulating an important point: In the case of an Alpha-1 antitrypsin deficiency, the possibility of accompanying bronchiectasis should always be considered.
Bronchiectasis
Bronchiectasis, or bronchiectasis, is defined as a chronic widening (dilatation) of the bronchi and the smallest bronchioles as a result of destruction of the muscles and elastic connective tissue.
The term bronchiectasis is composed of "bronchi" and "ectasia." Bronchi are the lower airways in the lungs. This hollow respiratory system consists of progressively smaller branches. In medical terminology, ectasia refers to the usually spindle-shaped, sac-like, or bladder-like dilation of a hollow body or vessel. Bronchiectasis is therefore also described as dilation or outpouching of the lower airways.
Symptoms
The symptoms are characterized in particular by the accumulation of secretions in the bronchiectasis and the resulting bronchial infections:
- chronic cough
- Sputum (often slimy, discolored)
- Blood in sputum
- rattling noises
- repeated infections, characterized by an increase in coughing and sputum production beyond the usual level
- Infections with rare, unusual germs
- Chest pain
- Shortness of breath
- severe fatigue
- Acute worsening of symptoms (exacerbation), sometimes several times a year
The most common symptoms are a cough with large amounts of mucus and sometimes purulent sputum, followed by shortness of breath or difficulty breathing. The severity of the symptoms varies greatly from person to person.
Good to know: Only the presence of symptoms makes bronchiectasis a bronchiectasis disease.
frequency
Bronchiectasis is a heterogeneous chronic lung disease that can be associated with many different conditions, such as asthma, COPD, and pulmonary emphysema.
The role of bronchiectasis caused by alpha-1 antitrypsin deficiency (AATD) is not yet fully understood. According to the bronchiectasis registry, it is currently estimated that 1 to 5 % patients with bronchiectasis occur within the AATD cohort. However, the cause of bronchiectasis is unknown in 36 % patients within the registry, among whom there may be additional patients with AATD. The most clinically relevant form in AATD is the PIZZ genotype, the homozygous form. Both PIZZ and the gene variant null are associated with a high risk (80 to 100 %) of developing pulmonary emphysema.
A study with exclusively substituted AATM patients documents that 18 % of the participants have bronchiectasis, which is presumably related to the severity of the disease.
Diagnostics
In addition to diagnostic procedures such as pulmonary function testing, laboratory tests (e.g., inflammatory markers, eosinophils), and cytological examination (e.g., of sputum), bronchoscopy and high-resolution computed tomography (CT) are considered the method of choice for basic diagnostics. X-ray examinations are insufficient for diagnosis.
Bronchiectasis is diagnosed in CT scans based on the following criteria:
- bronchoarterial ratio ≤ 1 (the bronchus must be larger than the arteries)
- Lack of bronchial narrowing with a distance from the visceral pleura ≤ 1 cm (dilated bronchi that reach almost to the chest wall, to the pleura)
Good to know: If no CT scan has been performed in the past two years, a computed tomography scan should be carried out if bronchiectasis is suspected. In addition to the radiologist, a pulmonologist should also review the CT results, as bronchiectasis can sometimes be missed.
Take advantage of the tiered diagnostic options offered by your family doctor, pulmonologist, and bronchiectasis centers. Don't hesitate to ask for a referral, and be aware that in acute cases, appointments with specialists and centers can be arranged quickly.
Therapy goals
- Treatment of the underlying disease
- Improvement of mucociliary clearance
- Prophylaxis of infections (e.g. vaccination)
- Treatment of the infection
- Treatment of airway obstruction
- Treatment of chronic inflammation
Mucociliary clearance (self-cleaning mechanism)
The primary function of the respiratory tract is to process the air we breathe, primarily humidifying, warming (mainly in the mouth and nose), and cleaning it. The inhaled air is then processed in the alveoli at the ends of the bronchi to optimize oxygen exchange. The lining mucous membrane plays a crucial role in filtering the inhaled air. With each breath, minute dust particles, droplets, and even pathogens such as bacteria, viruses, and fungi can enter the respiratory tract.
Important to know: Bronchiectasis can disrupt the natural self-cleaning mechanism of the respiratory tract mucosa.
The increased accumulation of mucus in the bronchial diverticula provides an ideal breeding ground for bacteria and is a source of frequently recurring infections. This bacterial colonization includes not only "normal" bacteria, but also pathogens such as Pseudomonas aeruginosa and Streptococcus pneumoniae (pneumococci), which can trigger serious infections. Biofilms play a role here; these are microbial communities that adhere to the mucous membrane of the respiratory tract and form a tough, protective matrix. These biofilms can complicate the treatment of respiratory infections because they protect pathogens from the immune system and antibiotics.
Secretarial Management
Independent mucus management, aimed at first thinning the mucus and then removing it from the bronchi, supports mucociliary clearance and should be consistently applied. First, consult your pulmonologist, respiratory physiotherapist, or respiratory therapist to determine which of the various aerosol inhalation devices is best suited to your individual situation. Instruction in the correct use of the device is recommended. Respiratory physiotherapy plays a crucial role in the treatment of bronchiectasis.
Inhalation plan
Have your doctor create an inhalation plan for you to ensure that the therapy is used as effectively as possible.
An example of an optimal sequence for better understanding:
- First, inhale the fast-acting bronchodilator (e.g., salbutamol, Atrovent). Wait 5 minutes after inhalation.
- Then inhalation of the mucolytic (e.g., saline solution 0.9 %, 3 % or 6 %)
- The moistened secretions can now be removed more easily using respiratory therapy (as recommended by your therapist).
- Only then is the long-acting spray inhaled.
- Finally, if necessary, an inhaled antibiotic (if prescribed).
Talk to your doctor about patient education! The correct use of an inhaler must be learned. Aids can be used to mobilize and loosen secretions.
Infection prevention
Use vaccinations to prevent serious infections. For example, Streptococcus pneumoniae, or pneumococcus, is the most common cause of pneumonia. With the new pneumococcal vaccine CV20 (also known as PCV20 or Prevenar 20), only one vaccination is now required.
Ask your doctor about other recommended vaccinations for chronic respiratory and lung patients and their current vaccination status.
Staphylococcus aureus is a bacterium that is frequently found in damaged lungs. Its primary source is usually the teeth. From there, the bacterium migrates to the lungs. Therefore, you should visit your dentist for professional dental prophylaxis (teeth cleaning) at least once, ideally twice, a year – even if your health insurance doesn't cover these costs. Some statutory health insurance companies now cover at least a portion of the costs.
Patients undergoing long-term oxygen therapy often find it difficult to have their teeth professionally cleaned. If necessary, seek advice from a pulmonary clinic, which can refer you to a dentist for consultation.
Treatment of chronic inflammation
Antibiotics are used primarily to combat bacterial infections. Some antibiotics are effective against only a few specific types of bacteria, while others are effective against a wide variety of bacteria. Antibiotic prophylaxis is also an option.
A new drug is currently undergoing the approval process. Brensocatib is the first drug designed to treat the underlying cause of chronic inflammation. Approval is expected next year.
The use of so-called biologics is currently being investigated in studies of COPD patients with elevated eosinophil counts and will likely also be used in eosinophilic bronchiectasis. They represent a supplement to or alternative to the current use of cortisone.
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