Bronchiectasis Update - LPNI

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Bronchiectasis Update

LNPI Health Topic - July 2023

Bronchiectasis is a very debilitating chronic lung disease, first described by the French physician Rene Laennec over 200 years ago. In recent years, research has delivered some exciting breakthroughs particularly in cystic fibrosis (CF) bronchiectasis. Non CF bronchiectasis is also now coming in for focussed attention, with the first global conference in Hannover, Germany in 2016.

Bronchiectasis is the widening of the bronchial airways with permanent fibrotic changes (scarring). Cilia are damaged in the process, so coughing is often necessary to clear mucus/sputum from the airways. Definitive diagnosis of Bronchiectasis is with high resolution chest tomography (HRCT).

Many aetiologies lead to the bronchiectasis pathology: eg cystic fibrosis (CF), COPD, airway tumours, pneumonia, allergic bronchopulmonary aspergillosis (ABPA ), tuberculosis mycobacteria (TB), non tuberculosis mycobacteria (NTM), measles and pertussis, tracheobronchial obstruction, inhaled foreign body, primary ciliary dyskinesia (PCD), recurrent aspiration pneumonia, immunodeficiency such as IgG, IgM and IgA. Despite so many aetiologies being linked to bronchiectasis, still in about 40 per cent of people with bronchiectasis, the aetiology remains idiopathic. This highlights the need for continued research.

After the first world conference in Hannover the European respiratory society (ERS) and the European lung foundation (ELF) have continued to work partnership with European multicentre bronchiectasis audit and collaboration (EMBARC). They deliver excellent webinars with a focus on people with bronchiectasis and health professionals. Another great opportunity to enhance your bronchiectasis knowledge is at the 6th world bronchiectasis and NTM world conference in New York NY, USA, July 18-20, 2023. https://web.cvent.com/event/efc57d59-8b0f-4d84-ae8c-0a86075dff80/ EMBARC has also established a bronchiectasis registry across Europe. People with bronchiectasis are being recruited to the registry for more successful clinical trials and strategies for personalised medicine. https://openres.ersjournals.com/content/2/1/00081-2015

The Lung Foundation Australia (LFA) has started to implement a bronchiectasis registry in Australia endeavouring to replicate some aspects of the EMBARC registry.

https://lungfoundation.com.au/research/our-research/bronchiectasis/

In the USA, a bronchiectasis and NTM research registry has been established.

https://www.bronchiectasisandntminitiative.org/Research/Registry/Bronchiectasis-and-NTM-Research-Registry LFA has developed the Bronchiectasis Toolbox. This is a website useful for people with bronchiectasis and health professionals. It also has an Indigenous section covering the Aboriginal peoples of Australia and Maori peoples of New Zealand. https://bronchiectasis.com.au/

Aboriginal people in Central Australia have high rates of pneumonia and recurrent chest infections. Sadly, this can lead to bronchiectasis and to early mortality, with a mean age of death 46.3 years as opposed to non Indigenous people in Melbourne, Australia, mean age of death 70.5 years¹. A retrovirus amongst Aboriginal people in Central Australia, HTLV-1 (human T-cell lymphotropic virus-1) has a significant association with Bronchiectasis². This association has also been identified in the United Kingdom².

In recent years there have some exciting global developments in CF bronchiectasis. The mutation on the CFTR gene F508 delta that causes CF Bronchiectasis has been identified. This has led to treatment of CF bronchiectasis for many people. Just last year the medication Trikafta has been approved by the Australian government on the pharmaceutical benefit scheme (PBS) for people with the this gene mutation. https://www.health.gov.au/ministers/the-hon-greg-hunt-mp/media/landmark-pbs-listing-for-australians-with-cystic-fibrosis

Currently there is a global Insmed Aspen phase 3 trial, targeting non CF bronchiectasis with a medication Brensocatib that inhibits neutrophil activation. https://www.prnewswire.com/news-releases/insmed-announces-initiation-of-phase-3-aspen-study-of-brensocatib-in-patients-with-bronchiectasis-301183121.html

Great to see this global effort and energy targeting bronchiectasis. Hope all these links and references are helpful in your practice.

Erik Tikoft BNg, RN, Respiratory CNC (Retired), Alice Springs Hospital, NT, Australia

References

Primer-Bronchiectasis, Nature Reviews, Disease Primers, Article citation ID (2018) 4:45

American Lung Association, Learn About Bronchiectasis, last updated, 17/11/22

UpToDate, Clinical manifestations and diagnosis of bronchiectasis, last updated 18/1/23

1 Sean Blackhall et al, Poster, TSANZ ASM, Gold Coast, QLD, Australia, March 2015

2 UpToDate, Human T-lymphotropic virus 1:Disease associations, diagnosis, and treatment, Updated

9/3/22