Insights into molecular and clinical characteristics of very early systemic sclerosis

Abstract

Purpose of reviewThe early heterogenous presentation of systemic sclerosis (SSc), in particular without skin involvement, has been a confounding factor delaying early diagnosis. In fact, early signs of SSc as Raynaud's phenomenon and puffy fingers, are also typical of other connective tissue diseases (CTDs) such as mixed CTD and undifferentiated CTD. In the last decade, a significant effort has been dedicated in defining molecular characteristics that could be used as early SSc biomarkers. In this narrative review, we address the present situation where several clinical scenarios are in search of a correct positioning into the prescleroderma (pre-SSc) phase as well as in the very early phase of SSc. Recent findingsLiterature data showed that a part of patients classified as sine scleroderma SSc (ssSSc), mixed CTD and undifferentiated CTD may already belong to the very early phase of SSc, thus having a different pattern of progression to SSc. Recently, the very early diagnosis of systemic sclerosis (VEDOSS) criteria has been validated. Summarywhile the area of pre-SSc still remains fuzzy, the VEDOSS study has shown that a 'window of opportunity' does exist also for SSc. In the very next future, this may allow to start the treatment to prevent the disease progression to a more advanced fibrotic stage.

Increased Circulating Soluble Junctional Adhesion Molecules in Systemic Sclerosis: Association with Peripheral Microvascular Impairment

“…Based on the present data, we are aware that further studies are necessary to determine if serum levels of both sJAM-A and sJAM-C may correlate with additional clinical SSc manifestations. Furthermore, longitudinal studies monitoring changes in levels of sJAM-C in patients with a very early diagnosis of SSc [63] who progress into an established SSc disease will help us to gain further insights into the potential diagnostic value of this circulating molecule. Prospective follow-up analyses of SSc patients are also required to fully unravel whether sJAM-C might be suited as a risk biomarker for the development of new DUs, as well as DU recurrence and/or refractoriness to treatments.…”