Study explores the link between vasculitis and bronchiectasis in people with alpha-1 antitrypsin deficiency

Background

Alpha-1 antitrypsin deficiency (AATD) is an inherited condition that can cause emphysema, a form of COPD (chronic obstructive pulmonary disease). It has also been linked with liver disease and autoimmune conditions. These include a group of diseases called vasculitis, which cause inflammation and damage to small blood vessels. Vasculitis can affect different parts of the body, including the kidneys, digestive system and lungs.

Although both alpha-1 and vasculitis affect the lungs, there has been limited research on symptoms in people who have both conditions.

What did the study look at?

A new study compared people with alpha-1 who also had vasculitis with those who did not.

Researchers used information from the European Alpha-1 Research Collaboration (EARCO) registry. The registry was created after patients highlighted the need for more research into alpha-1. It collects information about alpha-1, how it develops and factors that may affect people’s health.

What do the results show?

The study found that vasculitis was rare in people with AATD. However, those with vasculitis were more likely to have bronchiectasis, a condition in which the airways become permanently widened, leading to mucus build-up and repeated infections.

The results show that:

• lung disease was common among people with AATD, regardless of whether they had vasculitis
• COPD was much less common among people with vasculitis
• bronchiectasis was reported in over half (50%) of people with vasculitis compared with less than one in four people (21%) without vasculitis.
• However, people with vasculitis did not have worse emphysema symptoms or poorer lung function.

There are several possible explanations for the association between vasculitis and bronchiectasis in people with AATD.

AATD can damage airways, make it harder to clear mucus and increase the risk of infections. It is also linked to inflammation and immune system problems. These are also common features of bronchiectasis.

Only a small number of people in the registry had vasculitis (16 people), reflecting how rare the condition is. This means that the results should be treated with caution, and larger samples of patients will be needed to understand more about the findings and the reasons for them.

Why is this important?

The only treatment currently available for alpha-1 is called augmentation therapy. This is where alpha-1 antitrypsin (AAT) is delivered through a drip, usually in a hospital or clinic. Not all countries offer this treatment, as it is expensive and inconvenient for patients, or they may only offer it in the most severe cases. This means large numbers of people are receiving no treatment despite having symptoms of the disease.

Researchers are looking for more affordable treatments for less severe forms of alpha-1. Studies using registry data can improve understanding of alpha-1, which may help support earlier diagnosis and better care in the future.

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Read the full paper in the European Respiratory Journal: https://publications.ersnet.org/content/erj/early/2026/09/07/1399300300779-2026