The critical window of very early systemic sclerosis: a framework for a specialized early systemic sclerosis clinic

Abstract

Background
This study, led by the Egyptian Society for Microcirculation in Rheumatic Diseases, aimed to address the oversight of very early systemic sclerosis (SSc) as a pre-systemic sclerosis stage, hypothesizing that current clinical practices primarily focus on diagnosing SSc only when it meets established classification criteria. Identification of this patients’ cohort at such an early phase is a window of opportunity to treat those at high risk of skin fibrosis or organ involvement before such events occur.


Objective
The aim of this work was to establish a standardized, multidisciplinary clinic dedicated to the early assessment, diagnosis, and management of patients with very early systemic sclerosis (veSSc).


Methods
The methodology adhered to the PRISMA guidelines for reporting systematic reviews. To develop the clinical care standards framework, key clinical questions were systematically formulated using the PICO framework (Population, Intervention, Comparison, Outcome); a comprehensive literature review was conducted from 2010 to 2025 across multiple databases, and a geographically diverse task force of national experts was assembled to participate in a two-round Delphi process to assess consensus levels.


Results
Twelve key clinical questions were systematically developed using the PICO (Population, Intervention, Comparison, Outcome) framework. Following a review of the literature, and with the endorsement of the Egyptian Society for Microcirculation in Rheumatic Diseases, a Delphi process was carried out. Consensus was reached (i.e. ≥ 75% of respondents strongly agreed or agreed) on the wording, the grade of recommendation, and the level of evidence of all the clinical standards identified by the scientific committee.


Conclusion
Setting up a very early systemic sclerosis (veSSc) clinic involves establishing a system for early detection and management of SSc, focusing on patients with Raynaud’s phenomenon and other early symptoms. The goal is to identify individuals at risk of progressing to SSc and offer prompt, targeted interventions.