Very early and early diagnosis of systemic sclerosis

Systemic sclerosis (SSc) is easily diagnosed when the disease is evolved to skin fibrosis with obliterative vasculopathy and internal organs involvement. In the very early/early phase of SSc, the diagnosis remains very difficult because of the lack of validated diagnostic criteria. Actually, the American College of Rheumatology (ACR) and LeRoy classification criteria are not sufficiently sensitive to enable very early/early diagnosis of the disease, limiting the possibility of a precocious treatment. Therefore, in many cases, treatment is delayed for several years following the onset of Raynaud‘s phenomenon (RP) and even after the onset of the first non-RP symptom. For this reason, the very early/early diagnosis of SSc is today of pivotal importance. Recently, RP, puffy fingers turning into sclerodactyly and antinuclear antibody (ANA) positivity are considered the three red flags for the suspicion of a very early SSc. Further signs such as positivity of other specific autoantibodies and/or videocapillaroscopy pattern may allow very early diagnosis of SSc. This may allow follow-up of the patient and start the appropriate therapeutic regimen to arrest the disease progression when an organ involvement is detected. The time gap between the onset of signs and the diagnosis can be considered the ‘window of opportunity‘ where the disease may be stopped before skin and internal organs are irreversibly damaged. Therefore, the main topic discussed in this chapter will be ‘why we need to distinguish between a very early and an early diagnosis of SSc‘.