Very early diagnosis of systemic sclerosis

Systemic sclerosis (SSc) is an incurable chronic autoimmune disease associated with high morbidity and mortality. The current validated or proposed criteria are not appropriate to make a very early diagnosis of SSc. This implies that the diagnosis of SSc and, consequently, an appropriate therapy are delayed until the appearance of skin involvement and/or clinically detectable internal organ involvement when microvascular remodeling, tissue fibrosis, or atrophy are already irreversible. In a recent Delphi exercise, 4 signs/symptoms have been identified as necessary for the very early diagnosis of SSc: Raynaud’s phenomenon (RP), puffy swollen digits turning into sclerodactily, antinuclear antibodies and specific SSc antibodies (anticentromere and antitopoisomerase‑I antibodies), and abnormal capillaroscopy with scleroderma pattern.
Patients with very early SSc are the target of the recently launched the VEDOSS program, which has been designed to diagnose SSc very early and to examine whether this may change the disease prognosis. Although patients with RP, autoantibodies, and SSc capillaroscopic pattern could be easily followed up, there is still no agreement on the predictors that may allow us to identify patients who will develop an established disease.