Bronchiectasis is caused when the airways in the lungs permanently widen because of damage caused by inflammation and infection. This widening makes it difficult for the lungs to remove mucus. The mucus lining the lung is the key way particles and bacteria are cleared from the airway tubes. As the mucus builds up, it becomes easier for bacteria to multiply and infect the lung system.
It’s a serious disease that is only growing in prevalence; one study estimated that in the UK alone, the incidence of the disease grew from 350 per 100 000 in 2004 to around 566 per 100 000 in 2013. Across the Atlantic, around 139 in 100 000 US adults have the disease.
Despite this, clinicians still do not fully understand the disease’s causes, levels of severity, and treatment options. There have been hundreds of studies on how to treat bronchiectasis across the world, yet the results are often too different to say with confidence which treatments work, and why.
In a recent paper published in The Lancet Respiratory Medicine, researchers used an international clinical research network to describe the clinical characteristics of bronchiectasis and compare the disease between different European countries.
The authors are part of the iABC project, which is funded by the Innovative Medicines Initiative (IMI), and is helping to develop two inhaled antibiotics for patients with either bronchiectasis and the related disease cystic fibrosis.
“There's a whole bunch of diagnostic labels that lead to bronchiectasis,” says Professor J Stuart Elborn of Queen’s University Belfast who is also one of the paper’s authors.
He and the co-authors wanted to get a continent-wide comparison of its characteristics, which could then help them explore possible differences in clinical outcomes. “We have studies now that will help us get to the answers and really dig deep into this,” said Prof. Elborn
To do this, they turned to the European Bronchiectasis Registry (EMBARC), which the project also co-developed. Since 2015, bronchiectasis patients across Europe and Israel can give their informed consent for the registry to collect their data for analysis for up to five years. For example, the registry has information on patient demographics, comorbidities, lung function, exacerbations, and the disease’s impact on patient quality of life, to name a few.
Using statistical analyses, the researchers examined the characteristics of almost 17 000 people with bronchiectasis, revealing how the disease manifests itself in Europe.
The results showed that the most common identified cause of the disease was a ‘post-infective disease’; when patients contracted an earlier infection or disease that later caused bronchiectasis.
Bacterial infections causing the disease were most common in Southern Europe; Pseudomonas aeruginosa was prevalent in over 50% of cases, perhaps owing to the bacteria flourishing in warm, damp environments.
Perhaps most the most revealing result in the study is that most severe cases of the disease seem to happen in countries in central and eastern Europe, such as Poland, Ukraine, and Bulgaria. One reason this might be the case is that public healthcare in these countries is not geared towards early detection of the disease, meaning it has time to worsen before being treated.
Prof. Elborn says that this study helped to reveal the patterns of bronchiectasis infection in Europe, and is one of the first major results from the EMBARC platform since it launched.
He predicts that this data will easily generate many future project papers that will further paint a more detailed picture of the disease in Europe. He is also cautiously optimistic that with EMBARC, things are turning in the right direction to treat this disease.
“It's now got scalable funding from European respiratory society and pharma companies who are really keen to develop this particular area,” he said. “From that perspective, the investment from IMI has moved the field on enormously.”
“There's at least 10 clinical trials going on at the moment in bronchiectasis supported by the registry, many of which have been supported also by samples that we collected during the trials that were undertaken in the IMI programme,” he said.
iABC is supported by the Innovative Medicines Initiative, a partnership between the European Union and the European pharmaceutical industry. It is also part of the antimicrobial resistance (AMR) programme New Drugs for Bad Bugs (ND4BB).