From Raynaud's Phenomenon to Very Early Diagnosis of Systemic Sclerosis- The VEDOSS approach

Systemic sclerosis (SSc) is a heterogeneous chronic autoimmune disease that is very difficult to diagnose in the early phase, because of the low sensitivity of the classification criteria currently used to identify patients without skin involvement, with an important delay on the therapy that is often started when internal organ involvement has already irreversible. The biggest challenge in the fight against SSc is to detect valid predictors of disease so as to treat patients since the earliest stages of disease. Raynaud's phenomenon (RP), antinuclear antibodies (ANA) positivity, and puffy fingers have been recently indicated as the "red flags", the main elements to suspect SSc and then to perform further tests to confirm the diagnosis in particular nailfold video capillaroscopy and evaluation of specific disease antibodies (anti-centromere and anti-topoisomerase I). Particularly, RP is the earliest, even if aspecific sign of SSc, and more and more attention should be paid to its early identification in order to reduce the diagnostic delay. Besides, the time gap between the onset of RP and the diagnosis should be considered as a "window of opportunity" for SSc patients, through which the physician can act with effective drugs able to block or at least slow the progression of the disease.