Our aim was to analyze the concentrations of ELF components in VEDOSS patients either to understand its value in aiding very early diagnosis or to determine their potential diagnostic value.
30/114 SUBJECTS had primary Raynaud’s phenomenon (PRP); 54/114 were classified as VEDOSS (RP,Puffy fingers, ANA,SSc specific autoantibodies or SSc-specific capillaroscopic pattern) not fulfilling 2013 ACR/EULAR criteria (score < 9), while other 30 VEDOSS patients fulfilled new ACR/EULAR criteria despite lack of any sign of skin and internal organ involvement. ELF and its components correlated with age (p<0.05 for all). Logistic regression analysis using ELF variables identified a specific algorithm (SSc score) ranging from -3.92 to 5.77. The score showed good ability to discriminate between patients with SSc and VEDOSS patients already classified as SSc when compared to VEDOSS patients not yet classified as SSc (AOUC under ROC curve: 0,853 (95%CI 0,797-0.910)). Within the VEDOSS database, patients fulfilling SSc classification criteria, had average score of 0.44 vs -0.86 of patients not fulfilling the criteria, even after correcting for age (p=0,026). SSc score showed a fair ability to discriminate between the two groups (AOUC = 0.756, 95%CI 0.646-0.865). Moreover, SSc patients had significantly higher SSc score compared to VEDOSS patients already classified as SSc, confirmed after age correction (p<0.000). No difference in SSc score has been observed between PRP and VEDOSS patients not yet classified as SSc.
Our data indicate that the SSc score is a simple serum test that can be used in patients with RP to aid in the very early diagnosis of SSc. Furthermore, the identification of VEDOSS patients with a high SSc score test and already classified as SSc even in the absence of internal organ involvement can be used in intervention trials aimed to prevent further disease progression