The spectrum of Systemic Sclerosis: what are we looking at?

Abstract

Systemic Sclerosis (SSc) is characterized by vascular inflammation, vascular hyperreactivity and excess tissue collagen deposition. These pathologic changes lead to macro and microvascular injury and to fibrosis of the skin and internal organs resulting in organ damage. The exact mechanism and triggers for endothelial dysfunction and perpetuation of pathologic changes are only partially understood and still needing further research. Therefore, an early diagnosis is mandatory. The new SSc classification criteria (ACR/EULAR 2013) reflects the need to create an earlier treatment window of opportunity, based on a better understanding of the pathophysiology of the disease and recognizing the role of the specific SSc autoantibody profile in clinical manifestations and disease prognosis. SSc autoantibodies may “work” as predictors of a specific organ involvement, of prognosis and survival, in the several subtypes of the disease, historically just divided in limited and diffuse, based on the extend of skin involvement. Looking at a SSc patient the manifestations spectrum may be broad and change / evolve over the course of the disease with inflammation subsiding and fibrosis arising, resulting in irreversible organ damage. Unfortunately treatment options in SSc are still below expectations compared to other connective tissue diseases, and must be introduced according to disease manifestations and depending of aiming to target inflammation versus fibrosis. Thus, integrating the knowledge of these various aspects of the disease, rheumatologists can, in a more careful and targeted way, monitor and recognize potential internal involvement organ, as well as, potential complications and introduce treatment earlier. For this to be accomplished, it is important to perform regular patients follow-up and regular screening for specific organ involvement.