Alpha-1 antitrypsin deficiency (alpha-1) is a genetic disorder that can damage lung tissue. While alpha-1 is primarily associated with pulmonary emphysema, there is growing evidence that it may also contribute to the development of bronchiectasis. Both conditions are chronic, progressive, and inflammatory lung diseases.
This page explains what bronchiectasis is, its connection to Alpha-1, and what scientific findings exist on the subject.
Bronchiectasis is an irreversible widening („ectasis“) of the bronchi, often accompanied by thickening of the airways and pouches in which thick mucus can collect.
Bronchiectasis is diagnosed when the bronchi (B) is dilated relative to the accompanying artery (A), with a B/A ratio greater than or equal to 1. Symptoms such as increased mucus production, frequent coughing, recurrent infections, and progressive deterioration of lung function characterize this disease, which results from a chronic inflammatory response.
The bronchial walls, called cilia, can no longer properly remove mucus, which promotes the growth of bacteria and often leads to infections.
Bronchiectasis, along with asthma and COPD, is one of the most common chronic lung diseases – yet it remains largely unknown to the public.
This page focuses primarily on bronchiectasis in adults, which is referred to as non-cystic (non-CF) bronchiectasis.
In Germany, the prevalence is approximately 1:1,000 (F. RingshausenThis means that more than 100,000 people in Germany suffer from bronchiectasis, with women being slightly more frequently affected than men. The incidence of bronchiectasis increases with the age of the patients.
Bronchiectasis can be triggered by various factors, including:
In many cases, however, the cause remains unclear (idiopathic).
Typical symptoms of bronchiectasis include:
Bronchiectasis is usually diagnosed through a combination of:
The treatment of bronchiectasis pursues various goals, such as the prevention of infections in the chest cavity, the treatment of symptoms, the improvement of quality of life, and the prevention of a worsening of the disease.
With good treatment, it is possible for people with bronchiectasis to remain stable for many years and to control their symptoms well.
The one released in 2024 S2K GUIDELINE This describes the management of adult patients with bronchiectasis. The main pillars are:
Alpha-1 antitrypsin is a protein that protects the lungs from damaging enzymes such as elastase. A deficiency of this protective protein leads to uncontrolled tissue damage, which can promote not only emphysema but also bronchiectasis.
The protein deficiency leads to persistent (chronic) inflammation in the bronchi. The damaged airways are less able to clear mucus, resulting in repeated infections. Ultimately, without sufficient protection from alpha-1, the connective tissue in the airways weakens, promoting bronchial dilation.
The treatment of bronchiectasis in alpha-1 deficiency requires a combined approach consisting of the above-mentioned basic therapy for bronchiectasis and specific therapy for alpha-1 patients, such as alpha-1 substitution therapy.
Additional measures include quitting smoking, protection from environmental toxins, and regular check-ups.
Accompanying measures are also important, such as...
The information from [source] is highly recommended. ELEVEN Regarding bronchiectasis: Treatment options are explained in detail, and self-help instructions are provided. This information is available in German, among many other languages!
Furthermore, you can find information on the pages of Respiratory League, the Patient Library and the Lung Information Service , as well as on the company's website Insmed.
Specific information about bronchiectasis in children can be found here. https://www.improvebe.org/
Child BEAR Net (Children's Bronchiectasis Eeducation, Advocacy and Research NetThe bronchiectasis collaboration (COVID-19) aims to improve the care and clinical outcomes of children with bronchiectasis. It was founded in 2021 under the auspices of the European Society of Respiratory Medicine.
For patients with bronchiectasis, a register The PROGNOSIS registry has been established. Since 2015, treating physicians have been reporting patient data to the central office in Hanover. Patients with cystic fibrosis are not registered. More than 1,300 patients have now been recorded in this registry.
EMBARC stands for European Multicentre Bronchiectasis Audit and Research Collaboration.
It is a Europe-wide research network that collects data on bronchiectasis in order to better understand its causes, progression, and optimal treatment strategies.
EMBARC was founded in 2012 and is a network for scientists and clinical experts. The central focus of this project was the establishment of the European Bronchiectasis Registry. The EMBARC Registry is open to all researchers across Europe who care for patients with bronchiectasis.
On the following page you will find physiotherapists who specialize in cleaning the airways (secretion mobilization):
