Document resource
Introduction Context: The VEDOSS (very early diagnosis of systemic sclerosis) criteria were developed to identify established systemic sclerosis (SSc) at a very early stage. Several studies applied these criteria inconsistently, often using them to identify patients at a pre-disease stage. This has generated confusion between VEDOSS and the very preceding phase which could be defined as pre–scleroderma (pre-SSc), i.e. patients with clinical and biological signs that may be at risk of proceeding toward the VEDOSS phase and/or SSc as defined by ACR/EULAR criteria. Therefore, a consensus definition of pre-SSc is needed.Objective : to review and map existing definitions of pre-SSc and VEDOSS in the literature, to identify item combinations used, and to capture conceptual variations.Material and Methods Following PRISMA-ScR guidelines, PubMed and Web of Science were searched using the terms VEDOSS OR ‘very early diagnosis of systemic sclerosis’ OR ‘pre-scleroderma’ OR ‘pre-systemic sclerosis’ OR ‘pre-SSc.’Results Of 106 records screened, 40 full texts were included (8 on pre-SSc and 32 on VEDOSS). Most articles (80%) originated from Europe. Publication dates showed that the frequency of use of the concept of pre-SSc was progressively abandoned while VEDOSS was increasingly used. Three distinct definitions of pre-SSc were identified, alongside 13 different applications of VEDOSS criteria. Although 62.5% of studies cited the 2011 VEDOSS consensus, only 31.3% applied the complete definition incorporating the three step-1 essential items (Raynaud’s phenomenon AND puffy fingers AND anti-nuclear antibodies) and step-2 confirmation tools (SSc-specific autoantibodies and/or capillaroscopy). Twelve other combinations of the VEDOSS criteria were used to define ‘VEDOSS’ patients, demonstrating the inconsistency in the application of the 2011 criteria. Although pre-SSc and VEDOSS patients are generally not expected to present with SSc-related visceral complications, several articles reported internal organ involvement, ILD being reported in up to 35.5% of VEDOSS patients.Conclusions This scoping review highlights a substantial variability in the definitions of established VEDOSS and pre-SSc. The absence of a consensus definition for pre-SSc contributes to overlapping and blurring boundaries between these concepts. A consensus effort is needed to select a core set of items for a standardized definition of pre-SSc. Therefore, an updated SSc nosological framework should include a well-defined phase of pre-SSc (i.e. patients at risk of developing the disease) which will be then followed by VEDOSS and/or definite SSc according to 2013 ACR/EULAR criteria. In the future, each phase should be accompanied by appropriate interventions, outcome measures, and surrogate markers.