Find out what makes Sian happy
Sian is busy making her wedding plans
Get the latest news on Scleroderma and Raynaud's research projects. Find out how SRUK and medical institutions around the world are seeking out improvements in treatment and ultimately, working towards towards a cure.
We invested £75,000.00 into a project looking at identifying heart involvement in systemic sclerosis.
The study highlights the need to identify patients at risk of heart disease to offer them monitoring that could save lives by the early detection and treatment of life-threatening heart rhythm abnormalities.
Scleroderma can affect the heart in many ways - disturbing the conduction system controlling heart rhythm or compromising the heart muscle, valves, and the heart's external lining. Despite the fact that heart involvement is common in scleroderma, many patients do not experience any heart symptoms at all, leading to a potentially dangerous situation of unmonitored heart disease.
"We know that cardiac involvement in systemic sclerosis is associated with a very poor prognosis, accounting for between 14 and 55 percent of deaths among patients with systemic sclerosis," said study lead author Dr. Lesley-Anne Bissell from the Musculoskeletal Biomedical Research Unit at the University of Leeds. "Early diagnosis and treatment to reduce the risk of complications is therefore essential and crucial for a positive outcome."
To date the project has identified heart rhythm abnormalities in 15 patients, which will allow them to be carefully monitored and receive further potentially life-saving treatment.
The project is due to be completed July 2017 and a full update will be provided on how this project is helping to change lives.
We are committed to investing in research. We have invested over £10 million into Scleroderma and Raynaud's research to further understand the conditions, the mechanisms and progression to develop safer and more effective treatments as we work towards a cure. You can support our life changing work today by donating.
Derrett-Smith (UCL): “Molecular pathway analysis of keloidal morphea in systemic sclerosis"
Keloidal morphea is rare and therefore poorly understood, particularly when it develops in patients with co-existing scleroderma. Its development sometimes determines the decision to commence immunosuppressive drugs, though the rationale for doing so is not strong. The results from this study with patient samples will shed light on why some individuals develop keloidal morphea and the best ways to treat them.
Holmes et al (UCL): “The role of Endothelial-to-Mesenchymal Transition in scleroderma calcinosis"
Calcinosis occurs in up to 40% of scleroderma patients. Current therapies are limited and offer little benefit, because the biological processes that contribute to the development of calcinosis remain unknown. The endothelial cells lining blood vessels in scleroderma may change their properties and express genes leading to calcinosis and inflammation, a process known as endothelial-to-mesenchymal transition. Our studies with cultured endothelial cells will seek to understand how this happens and whether we can block it.
Pain (Liverpool, Alder Hay): “Assessing inflammation in childhood scleroderma: comparison of imaging and skin examination"
The effects of scleroderma on a growing child can be harsh: skin thickening limits bone and soft tissue growth, which can lead to deformity, disability and disfigurement. Treatment is with drugs that suppress the immune system which is thought to be overactive in this condition and causing inflammation, but getting the dose right is important as the drugs can have unwanted side-effects. This project aims to identify better ways to measure skin inflammation and so treat children appropriately.
Pauling et al, University of Bath: “An Epidemological Study of Systemic Sclerosis and its association with Cancer in the UK using the Clinical Practice Research Datalink (CPRD)"
This study will examine healthcare information from nearly 20 million UK residents and provide up to date figures on the number of patients in the UK with scleroderma whether the number of new patients with this disease is rising or falling. We will also examine the relationship between scleroderma and cancer to understand whether there is any link between the two conditions.
This was a project funded by Scleroderma & Raynaud's UK (SRUK). It was conducted by Xiaoyan Pan and colleagues at Hope Hospital, Salford (published in Scleroderma News, November 2014)
In the 'diffuse' form of scleroderma, skin thickening can progress fairly rapidly, usually starting in the hands, face or feet but then spreading upwards to involve upper arms, thighs, or trunk. Those affected are also at high risk of developing lung, kidney or heart involvement especially in the first three years of the illness. A number of different drugs with effects on the immune system (called 'immunosuppressants) are currently used by doctors in the treatment of early diffuse scleroderma. However, all can have side effects and the efficacy of the different drugs is not fully established.
The objective of the European Scleroderma Observational Study (ESOS) is, by careful recording and analysis, to examine the effectiveness of different immunosuppressant drugs currently favoured by doctors in treating scleroderma. Information obtained through ESOS will, in the future, help to inform doctors' treatment decisions.
ESOS is supported as part of the EULAR (European League Against Rheumatism) Orphan Disease Programme which aims to further knowledge of why scleroderma develops, and how best to treat scleroderma. To allow enough patients to be recruited into the study to provide meaningful results, ESOS opened 44 recruiting centres in 16 European countries between 2010 and 2012.
The approval processes for the different countries took longer than planned, with the result that recruitment was initially under target. The study protocol was therefore amended and opened another six recruiting centres in Austria, Canada and USA. ESOS was also publicised at international conferences to facilitate the recruitment. Despite this, a 12-month extension of recruitment was essential to hit target recruitment.
Funding from SRUK allowed the ESOS recruitment period to be extended by one year. Target recruitment was then completed, with 322 patients recruited!
Sian is busy making her wedding plans
A pioneering new mobile testing clinic will allow Leeds residents to undergo screening for two little-known conditions.
Find out what's happening for World Scleroderma Day on 29th June.