Author
Marion Wilkens, as appeared in Alpha1 Journal 2/2025.
Bronchiectasis refers to a persistent widening of the airways (bronchi) with impaired self-cleaning mechanisms of the lungs. This leads to persistent mucus production, chronic cough, recurrent infections, inflammation, and often, over time, a deterioration of lung function.
Alpha-1 antitrypsin deficiency (AATD), like other causes, can play a contributing role in the development of bronchiectasis. If the body lacks sufficient amounts of the protective protein alpha-1 antitrypsin, lung tissue is significantly more susceptible to damage from the body's own enzymes (e.g., neutrophil elastases). Over time, this can weaken the bronchial walls, impair the clearing of accumulating mucus from the airways, and ultimately contribute to the development of bronchiectasis.
For patients with AATTM, this means: Bronchiectasis is not uncommon, but a possible consequence – and should therefore be investigated by a doctor at an early stage if symptoms such as chronic cough, increased sputum production or frequent infections occur.
Why bronchiectasis in AAT deficiency deserves special attention
The typical symptoms – cough, sputum production, shortness of breath, and susceptibility to infections – often overlap with those of "conventional" COPD, asthma, or pulmonary emphysema. The underlying cause (e.g., an autoimmune thyroid disease) is therefore frequently overlooked. By the time bronchiectasis is diagnosed, the structural changes are usually already irreversible. Early detection can help slow its progression through appropriate therapies, physiotherapy and respiratory therapy, infection prophylaxis, and regular checkups.
For people with AAT deficiency, there is an established, specific therapy: replacement therapy, which can slow the progression of pulmonary emphysema and theoretically also help to limit bronchial damage. Therefore, it is very important that affected individuals and treating physicians keep bronchiectasis in mind as a possible complication of AAT deficiency and not only assume classic emphysema or COPD scenarios.
New developments: Drug approval for bronchiectasis
A major advance for the treatment of bronchiectasis, including for patients with AATD, is the recent approval of the first specific drug: The orally administered drug brensocatib (trade name BRINSUPRI®) was approved in November 2025. European Commission approval for the treatment of non-cystic bronchiectasis (NCFB) received. This is therefore for the first time an approved, causal therapy option in fibrosis bronchiectasis for many affected individuals.
The approval applies to patients aged 12 years and older with at least two exacerbations (infection-related or worsening episodes) in the previous year. In studies – including the large-scale phase 3 ASPEN trial – brensocatib demonstrated an approximately 201-fold reduction in the annual exacerbation rate, longer time to the next episode, and a slower decline in lung function. This drug could therefore be a valuable new option, especially for people with severe disease, frequent infections, or limited responses to standard therapy. For people with AATD and concurrently diagnosed bronchiectasis, this approval offers hope – naturally, the treatment decision must be made individually by the doctor and patient.
Since this topic affects many Alphas, we have launched a new patient information leaflet on AATM and bronchiectasis.
Visit our new Information page, which specifically deal with the topic of „Alpha-1 & Bronchiectasis“ for those affected, relatives and interested parties This section deals with the topic. There you will find clearly presented explanations, background information and helpful advice on diagnosis and therapy.
The content of this page is currently being reviewed by Dr. Pontus Mertsch. We are pleased to announce that he will soon be reporting on new developments – especially important at a time of significant progress due to regulatory approvals and increased research. Particularly valuable: On this page, you will find practical tips on how to better manage AAT deficiency and the associated risk of bronchiectasis, as well as how to effectively plan treatment and preventative care.
Therefore, it is important that patients, relatives and treating physicians inform themselves now and think together about an optimal therapy plan.
If you or a close relative suffers from AAT deficiency: Please note that Bronchiectasis is a frequently underestimated but serious complication. We can present this. The new information page from Alpha1-Germany and the approval of the drug Brensocatib open up new avenues – and we hope that you can benefit from them.
Stay vigilant, stay informed – and talk to your pulmonologist about options for diagnosis, treatment and prevention.
As of December 2025
