Reducing Uncertainty in the Progression of Lung Fibrosis in Scleroderma Patients

Having scleroderma often means living with uncertainty around how the disease will progress over time, and the effect that this could have on future quality of life. New research funded by SRUK aims to help address the impact of this ‘prognostic uncertainty’, by using indicators that will predict disease progression and ultimately improve treatment options.

If you have scleroderma, you may often feel unsure about how your condition and all its related symptoms might develop over time. It’s also common to worry about how its progression could impact your life in the future, particularly if you’ve been told that the condition will affect parts of your body like your heart or lungs. This has the potential to produce anxiety about your condition, in addition to confusion about when certain treatments might be necessary. As a result, this prognostic uncertainty can potentially have a profound effect on your quality of life and treatment.

However, by developing indicators that allow us to predict the progression of scleroderma and its symptoms, research can help reduce some of the uncertainty that surrounds the condition. Ultimately, such indicators could be one of the keys to enabling improvements to patients’ lives and treatment in the future.

New SRUK-funded research led by Dr Elisabetta Renzoni at the Royal Brompton Hospital and Imperial College London looks to assist in the development of an indicator that could help predict the progression of lung disease in people diagnosed with scleroderma. Specifically, the project aims to assess whether it is possible to establish a computer algorithm which would allow early CT scans of the lung to be used as a tool to predict the progression of lung fibrosis in scleroderma patients with interstitial lung disease (ILD). As a result, people living with systemic sclerosis (SSC) and ILD could soon be offered a better understanding of the likely progression of their lung fibrosis.

Lung fibrosis and scleroderma

ILD is an umbrella term for conditions that cause lung fibrosis, meaning the stiffening of the lungs by scar tissue that occurs to some degree in around half of people diagnosed with SSc. However, the extent to which symptoms develop over time varies considerably between patients. Whilst more than half of people living with SSc have some form of lung-involvement, only around one-third will experience increasing lung scarring, breathlessness and reduced survival chances as a result. As such, when someone is told that they have ILD, they often face extreme uncertainty about how the disease will progress and the impact of this upon their life.

The impact of uncertainty

Naturally, uncertainty about the prognosis of this condition can be a major catalyst of the anxieties that people living with scleroderma so often report that they experience. Worries about how or when the disease could deteriorate, and the health outcomes that this could cause, can seriously affect the mental wellbeing of patients and their loved ones. In turn, there is the potential for an individual’s quality of life to be affected, which can ultimately impact upon their general health, independence and enjoyment of everyday activities.

Furthermore, ‘prognostic uncertainty’ of this sort can also affect the ability to make effective treatment decisions. If the prognosis of a patient’s condition is unclear, this can restrict the doctors’ ability to determine which therapies they are likely to require and also when these will be needed. This limits the ability of doctors to manage a patient’s condition with the correct treatments before their symptoms worsen. As such, prognostic uncertainty does not merely generate concerns regarding mental health and quality of life, but also directly affects the treatment of individuals living with scleroderma.

New research: Reducing Uncertainty in Lung Fibrosis Progression

To tackle these issues and reduce the ‘prognostic uncertainty’ regarding the progression of lung fibrosis in scleroderma patients, there is an urgent need for better indicators that can accurately predict the likelihood of a patient’s fibrosis getting worse. These indicators can enable doctors to start effective treatments sooner and prevent irreversible scarring to the lungs, whilst also avoiding the use of potentially toxic treatments in patients who don’t need them.

As such, Dr Renzoni and her team at the Royal Brompton Hospital have set out to assess the possibility of establishing a computer algorithm that would enable early lung CT scans to predict the progression of ILD in scleroderma patients. During the study, which began in February 2022, they aim to assess archived chest CT scans of 500 patients with ILD, which were taken when they were first diagnosed. The team then plan to analyse long-term follow-up data of these patients and run a pre-existing algorithm that is already used in the treatment of patients with other types of lung scarring, in order to see whether it can accurately predict the progression of ILD in people with scleroderma.

They also plan to build on existing work in order to refine predictive algorithms specific to scleroderma, which they believe could have the capacity to predict the progression of lung fibrosis with even greater precision. The study will resultantly provide the basis for further research involving the UK’s other scleroderma centres, which will aim to ensure that the results of the study are applicable to all scleroderma patients with ILD and evaluate whether providing accurate prognostic information can increase the quality of life of individuals in practice.

As a result of this project and the research that could stem from it, people living with scleroderma and ILD could soon have a much more definite understanding of the prognosis of their condition, and may therefore benefit from multiple improvements to their quality of life and treatment. What is certain is that research of this sort - which enables earlier diagnosis, more precise treatment, and improvements to quality of life - is integral to tackling the condition and will be one of the keys to ensuring that nobody living with scleroderma has their life limited by the disease in the future.