Scoping literature review on applied definitions of very early diagnosis of systemic sclerosis (VEDOSS) and pre-systemic sclerosis in original articles

Context: The very early diagnosis of systemic sclerosis (VEDOSS) criteria were developed to diagnose systemic sclerosis (SSc) at a very early stage. However, several studies have applied these criteria inconsistently, often using them to identify patients at a predisease stage. This use has created confusion between the concept of VEDOSS and pre-SSc. With growing interest in predisease states in autoimmunity, a consensus definition of pre-SSc is mandatory. Objective: As a preliminary step towards such definition, this scoping review aimed to map existing definitions of pre-SSc and VEDOSS in the literature, identify item combinations used and capture conceptual variations. Methods: Following Preferred Reporting Items for Systematic Reviews and Meta-Analyses Extension for Scoping Reviews 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 (eight on pre-SSc and 32 on VEDOSS). Most articles (80%) originated from Europe. 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 and step 2 confirmation tools. Conclusion: This scoping review highlights the variability of the definitions of VEDOSS and pre-SSc that have been used in the literature. The absence of a consensus definition for pre-SSc contributes to overlapping and blurring boundaries between these concepts. A consensus effort is warranted to select a core set of items for a standardised definition of pre-SSc.