The VEDOSS Project – Predicting the Progression of Raynaud’s to Systemic Sclerosis

Can you imagine a world where science will enable us to accurately predict whether a person with Raynaud’s will progress to develop systemic sclerosis? The ability to do this creates the potential for patients to access earlier treatment to spare them from the worse aspects of the condition, and even inspires hope that the condition could be prevented from fully developing in the first place.

Recent research arising from the VEDOSS project aims to benefit those at risk of developing the condition in exactly this way, providing physicians with a tool that could be huge step forward in the prediction and early detection of systemic sclerosis.

The VEDOSS Study and Criteria

The VEDOSS Project is a collaborative, international research project developed to better understand the progression from Raynaud’s Phenomenon to systemic sclerosis and validate criteria proposed by the European League Against Rheumatism (EULAR) as ‘red flags’ which could assist in the early diagnosis of the condition. These criteria included Raynaud’s Phenomenon, puffy fingers, and the presence of autoantibodies which target molecules usually found inside of the cells of the body, in particular a type of autoantibody known as ‘antinuclear’ antibodies.

Patients with Raynaud’s phenomenon who experience two of three colour changes (white, red, or blue) affecting at least one finger of the hand were eligible to join the study. By 2018, 1150 patients who experience Raynaud’s phenomenon had been enrolled onto the VEDOSS database. From these patients, 553 were eventually included in this current research which aimed to identify a set of predictive factors capable of indicating whether an individual with Raynaud’s is likely to go on to develop ‘Systemic Sclerosis’ within a 5-year timeframe.

New Findings – An Effective Tool for Predicting Progression to Systemic Sclerosis

The analysis by the VEDOSS researchers, which included Professor Francesco Del Galdo from the University of Leeds, accordingly aimed to investigate the relationship between the presence or absence of each of these indicators (in isolation and in combination) in patients with Raynaud’s, and how this affects the likelihood of their progression to Systemic Sclerosis within a 5-year period. A better understanding of this relationship would allow the research team to develop a method which could predict which patients they see with Raynaud’s are likely to develop Systemic Sclerosis, and understand which early factors indicate the greatest risk of progression to the condition.

Promisingly, the analysis found that the presence of one or more of the indicators included in the VEDOSS criteria did indicate a higher risk of developing Systemic Sclerosis within 5 years. For example, 94.1% of patients in the study who had anti-nuclear antibodies and other systemic sclerosis-specific autoantibodies at outset, as well as puffy fingers, progressed to develop the condition. Conversely, patients lacking anti-nuclear antibodies at outset were far less likely to progress to systemic sclerosis within 5 years – only around 10% of these patients went on to develop the condition during the study.

The Importance of Prediction, Early Diagnosis and New Ways of Classifying Patients

The VEDOSS Criteria appears to offer valuable insight into the likelihood of a patient with Raynaud’s progressing to systemic sclerosis. But why is this so exciting to researchers and clinicians treating these conditions? As is the case with many progressive health conditions, the prognosis for those living with Scleroderma often looks most optimistic when diagnosis occurs at an early stage of progression. In these early stages, physicians have a window of opportunity to intervene with treatments which can slow progression and are therefore able to safeguard against the deterioration of a patient’s symptoms and quality of life. As a result, clinical tools like the VEDOSS criteria, which enable the detection of those at high risk of developing systemic sclerosis and those progressing into its early stages, are integral to the effective treatment of Scleroderma.

Furthermore, the VEDOSS criteria also provides an exciting opportunity to classify Raynaud’s patients in a novel way, based on the presence or absence of specific clinical features which directly indicate their risk of developing systemic sclerosis. As we better understand the causal links between factors like disease pathways and the onset and early indicators of the condition, there is potential that stratifying patients using methods like the VEDOSS criteria could enable targeted treatment to slow, or even halt, disease progression. This could allow clinicians to tailor treatment to individuals or specific sub-groups of patients, rather than using a one-drug suits all approach which can be a less effective method of treating a complex condition like systemic sclerosis.

Registries - The Need for Data in Raynaud’s and Scleroderma Research

What is clear is that studies such as these are dependent on large numbers of Raynaud’s and Scleroderma patients, and the ability of investigators to access and analyse data from a variety of patient groups in order to make these exciting findings. Researchers require a consistent flow of data from a large pool of patients to properly understand the early biological markers of Scleroderma, and how they link to the progression of the disease and other biological processes that play a role in the condition.

Access to data of this sort is therefore crucial to progressing understanding of the mechanisms that underpin Scleroderma, and how the condition can be treated in the future. Patient registries, which give researchers access to information about the condition of thousands of patients and how their condition evolves over time, could be the key to facilitating more exciting research into Scleroderma and Raynaud’s in the future.

Registries are also a valuable way for patients and researchers to work together to enable much of the exciting new research that is so integral to improving the lives of people living with Raynaud’s and Scleroderma around the world.