Systemic sclerosis (SSc) is a disease of blood vessels, skin and other organs. Around one in 10 patients with SSc develop a problem with the blood vessels in the lungs called pulmonary arterial hypertension (PAH). This causes breathlessness, heart failure and ultimately death. Around half of SSc patients diagnosed with PAH will die within three years. Better methods of detection are needed to allow earlier treatment, improve quality of life, and assist discovery of new treatments that target blood vessel damage.

Dr Thompson and his team at the University of Sheffield will use cutting-edge imaging methods to improve the identification of PAH in SSc (SSc-PAH). Patients will undergo magnetic resonance imaging (MRI) scans while breathing a special ‘hyperpolarised’ gas that provides detailed pictures of the transport of oxygen into the lungs from the air and can also measure blood flow in the lungs. Two groups of patients will be recruited; one group of patients with SSc-PAH and a second group of patients with SSc who have not yet developed PAH. Patients will have MRI scans, and some will have repeat tests after six months to assess disease progression. Patients will also have heart scans (echocardiography), breathing tests, CT imaging scans and a test to measure the blood pressure in the lungs.

The findings from this project have the potential to enable quicker and more accurate diagnosis, reducing the emotional and physical impact of this disease. It is also hoped that this will allow for better monitoring of PAH and how it responds to drug therapy in different patients, ultimately providing insight into how best to treat the condition.