Click here to see the findings of the survey
In December 2020, the PHA UK conducted an online survey to find out how interested people with pulmonary hypertension would be in a study aimed at diagnosing the condition earlier in those with systemic sclerosis.
Researchers are aiming to set up the study looking at the use of cutting-edge magnetic resonance imaging (MRI) scans and new disease markers in the blood to help PH be detected earlier. The technologically advanced MRI scans have recently been used in the diagnosis of lung scarring and researchers want to see if they can also be used for earlier diagnosis of pulmonary hypertension.
The survey was conducted in collaboration with Dr Ashraful Haque, Rheumatology Registrar and Clinical Research Fellow at the Royal Hallamshire Hospital.
The results of this survey show people with PH would be very interested in a study using MRI technology and new disease markers in the blood to detect PH earlier in those with systemic sclerosis. They would be willing to undergo additional scans and blood tests to support the study.
The findings from this survey will be used as evidence to support the application for approval and funding of the proposed study. The PHA UK will keep members updated with progress and opportunities to enrol in the study if approved. Thank you to everyone who shared their thoughts.
You can find out more about the proposed study below.
What is systemic sclerosis?
Systemic sclerosis is a condition where the body attacks different organ systems by forming antibodies. The condition is characterised by disease of blood vessels (‘vasculopathy’) and scarring of organ tissue (‘fibrosis’). Around 10% of patients with systemic sclerosis develop pulmonary hypertension.
Why does it take a long time to diagnose PH in someone with systemic sclerosis?
Existing evidence shows that early treatment of this condition, with specialist drugs we already have, can improve longer term outcomes. However, the diagnosis of pulmonary hypertension related to systemic sclerosis is often delayed because current screening measures (which include blood tests, echocardiography and breathing tests) are not good enough.
What will the study do?
Researchers are aiming to set up a study looking at the use of cutting-edge magnetic resonance imaging (MRI) scans and new disease markers in the blood to help PH be detected earlier. The technologically advanced MRI scans have recently been used in the diagnosis of lung scarring and researchers want to see if they can also be used for earlier diagnosis of pulmonary hypertension.
How would patients be involved in the study?
Patients with systemic scleroderma taking part in the study would have these MRI scans in addition to the current investigations they would undergo – which include blood tests, echocardiography and breathing tests and electrocardiogram (‘ECG’).
Those patients deemed at highest risk of PH would then undergo a CT (computerised tomography) scan of the chest and right heart catheterisation.
The blood test and MRI scan for the study would happen at the start of the study and would then be repeated after 12 months to look for changes that might signify pulmonary hypertension.
If you have any questions about the study, please contact researcher Ash Haque at firstname.lastname@example.org
Ash Haque is a rheumatology registrar with a specialist interest in connective tissue disorders such as systemic sclerosis. He is currently doing a clinical research fellowship on the Sheffield Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, and is very keen to undertake a PhD focussing on the earlier detection of systemic sclerosis related pulmonary hypertension.