World-first study into rare lung disease launched at Exeter

A worldwide first-of-its-kind study has launched at the Royal Devon University Healthcare NHS Foundation Trust, supported by the University of Exeter and the National Institute of Health and Care Research (NIHR), exploring treatment for a chronic condition affecting the lungs, causing inflammation and scarring.

Known as fibrotic hypersensitivity pneumonitis (FHP), people living with the disease experience symptoms such as difficulty breathing, weight loss, and a hacking cough that gradually gets worse over time. It is thought to affect 11 in 100,000 people, and there are around 2000 cases diagnosed in the UK each year.

FHP is a life-limiting condition that eventually leads to death, with median survival estimated to be around five to seven years after diagnosis.

It is hard to treat, and most patients are prescribed steroids to help with inflammation of the lungs, but this is without any evidence base to support this practice. The CHORUS study aims to find out how effective steroid treatments are (if at all), assess safety and potential harm, and provide formal guidelines to help improve how doctors can manage the condition.

Professor Michael Gibbons, Consultant Respiratory Physician at the Royal Devon, and Senior Investigator Fellow at the NIHR Exeter Biomedical Research Centre, University of Exeter said: “We’re delighted to launch this world-first study that will help us evaluate treatment for fibrotic hypersensitivity pneumonitis. CHORUS will not only deepen our understanding of the disease, it will provide crucial evidence that could reshape how we deliver care. Our hope is that this research will pave the way towards better, more targeted treatment options and ultimately improve the experience of those living with the condition, reducing the risk of complications that can lead to more hospital stays and more invasive interventions.”

For those affected by FHP, the study is a welcome step forward, offering potential for greater understanding and a clearer path towards improved therapies.

Garry Berry was diagnosed with FHP in May 2023 and shared his experience of living with the condition to help others. With the support of his family, Garry’s words are being published in his memory and in recognition of his wish to raise awareness of FHP and the importance of research.

“In 2021 I developed a persistent cough. After encouragement from my wife and children, I visited my GP and was referred for hospital tests. In May 2023 I was advised I had developed FHP, which I had never heard of before! After the specialist consultant explained what this lung condition was, that it would be progressive, it could not be cured and advised on the inevitable final outcome, I was rather taken aback.”

Garry, a representative on the CHORUS Patient Advisory Group and a layperson within the Trial Management Group, continued: “My FHP condition has now progressed to the stage where I am excessively gulping for air if I do anything at all strenuous, cough a lot of the time and am fatigued, even if I walk just a relatively short distance. But I still consider myself lucky.

“I still run our long-standing Planning and Architectural Practice, working about 60 per cent of my previous hours, and thankfully hasn’t limited me too much so far in this respect. An intense interest in both modern and historic cars and motor racing has been a feature of my life from a very young age, and while physical limitations mean I’ve had to cut back on events and volunteering, I have good friends and many cherished memories.”

The £2m CHORUS study, designed closely with patients and carers alongside EPIC-PF and Action for Pulmonary Fibrosis, will recruit participants from over 30 UK sites over two years.

It is delivered in partnership by the Royal Devon, the University of Exeter, the NIHR Exeter Biomedical Research Centre, Exeter Clinical Trials Unit, NIHR Exeter Clinical Research Facility and the University of Exeter’s Clinical and Biomedical Sciences department.

Garry said: “There has been little clinical research into the effective treatment of FHP. This is why the CHORUS Trial is so important, not just to find the effectiveness the current treatment, but also to raise awareness of FHP with clinicians and the wider public.

“If you are approached to take an active part in the CHORUS Trial, I wholeheartedly encourage you to strongly consider doing so. If my own FHP had not developed to a stage where I am excluded from the trial, I would be keenly participating. After all, if such important research is not undertaken, no progress will be made.”