medwireNews: Risk stratification using very early diagnosis of systemic sclerosis (VEDOSS) criteria can help identify individuals with Raynaud’s phenomenon who are most likely to progress to definite systemic sclerosis (SSc), study findings indicate.
Francesco Del Galdo (University of Leeds, UK) and co-investigators found that people with both SSc-specific autoantibodies and puffy fingers were most likely to develop SSc, whereas those negative for antinuclear antibodies (ANAs) were least likely to have the condition within 5 years of follow-up.
The VEDOSS registry includes 1150 patients with Raynaud's phenomenon and no history of SSc or other connective tissue diseases from 20 countries in Europe, North America, and South America between 2010 and 2018.
The current analysis included data for 553 people with at least one follow-up visit and a median time since the onset of Raynaud's phenomenon of 4 years.
At baseline, 73.7% of 544 participants with available data had detectable ANAs, one of the four VEDOSS criteria. Among the remaining three criteria, 39.5% of 527 patients had SSc-specific autoantibodies, 36.0% of 505 patients had nailfold capillaroscopy abnormalities, and 17.8% of 540 had puffy fingers.
Some participants had a combination of VEDOSS criteria, most commonly ANA positivity plus nailfold capillaroscopy abnormalities (29.3% of 509) and SSc-specific autoantibodies plus nailfold capillaroscopy abnormalities (15.6% of 514).
Del Galdo and team report that 254 participants completed the 5-year study of whom 52.4% progressed to meet the 2013 ACR–EULAR classification criteria for SSc.
The majority (94.7%) of the 133 progressors were ANA positive, corresponding to 58.9% of the 214 ANApositive patients at baseline.
Conversely, just 10.8% of the 37 ANA-negative participants met the criteria for progression.
Writing in The Lancet Rheumatology, Del Galdo and co-authors say that “[t]he absence of ANA at baseline was the factor most strongly associated with a lack of progression within 5 years.” Indeed, the risk for progression was a significant 82% lower when no ANAs were detected at baseline.
The researchers also looked at combinations of the VEDOSS criteria and created a risk scale according to the proportions in each group progressing to definite SSc. They found that patients with ANA, SScspecific autoantibodies, and puffy fingers at baseline had the highest progression rate, at 94.1% of 17, while those with ANA, SSc-specific autoantibodies, and nailfold capillaroscopy abnormalities had the second highest rate, at 82.2% of 45.
Del Galdo et al conclude that their data “confirm the clinical value of Raynaud’s phenomenon, puffy fingers, and ANA positivity among the pivotal signs and biomarkers to raise the suspicion of systemic sclerosis.”
They add: “These signs together with the positivity of systemic sclerosisspecific autoantibodies or an abnormal nailfold capillaroscopy, or both, might identify patients with Raynaud’s phenomenon who have a very early systemic sclerosis that is characterised by the highest risk to evolve to definite systemic sclerosis.”
In an accompanying commentary, John Pauling, from the University of Bristol in the UK, says the study “has provided valuable feasibility data, carefully charting the rate and nature of disease progression in VEDOSS patients that could influence cohort enrichment for future interventional studies [and thus] support preventative disease modification in systemic sclerosis.”
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Lancet Rheumatol 2021; 3: e834–843
Lancet Rheumatol 2021; 3: e820–821