Early Systemic Sclerosis: What’s in a Name

Current Treatment Options in Rheumatology, vol. 11

Abstract

Abstract Purpose of the review This review aims to clarify the different definitions and contexts of the term "early" in systemic sclerosis (SSc) and its implications in clinical practice and research. The term "early" has been used inconsistently, leading to confusion in diagnosing, managing, and conducting research on SSc. Recent findings Recent studies have explored the evolution of patients from very early SSc stages to established disease. The development of the Very Early Diagnosis of Systemic Sclerosis (VEDOSS) criteria has marked a significant step forward. However, studies have shown varying progression rates among patients meeting different "early" criteria, with a subset remaining in mild or indolent stages for extended periods. Significant gaps remain in predicting disease progression, prompting the need for more refined and personalized criteria. Summary Systemic sclerosis is a complex disease with high morbidity and mortality. The inconsistent use of the term "early" has led to challenges in diagnosis, treatment, and research. While the VEDOSS criteria have provided a framework for identifying very early SSc, further refinement is needed to improve disease progression predictions. A more personalized approach to patient monitoring and the application of adjusted VEDOSS criteria may enhance early intervention strategies, ultimately aiming to prevent disease progression in SSc.

Authors 4

1. Madelon C Vonk corresponding

   Radboud University Nijmegen · Radboud University Medical Center

   Affiliation as printed

   Department of Rheumatology, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands

2. Brigit E. Kersten

   Sint Maartenskliniek

   Affiliation as printed

   Department of Rheumatology, Sint Maartenskliniek, Nijmegen, The Netherlands

3. Jeska Kirsten De Vries-Bouwstra Aachen Department of Rheumatology, Leiden University Medical Center

   Leiden University Medical Center

   Affiliation as printed

   Department of Rheumatology, Leiden University Medical Center, Leiden, The Netherlands

4. Vanessa M. Smith

   Ghent University Hospital · Ghent University · VIB-UGent Center for Inflammation Research

   Affiliation as printed

   Department of Internal Medicine, Ghent University, Ghent, Belgium

   Department of Rheumatology, Ghent University Hospital, Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Centre (IRC), Ghent, Belgium