Systemic sclerosis - multidisciplinary disease: clinical features and treatment

Abstract

Systemic sclerosis is a chronic autoimmune disease of still not fully understood pathogenesis. Fibrosis, vascular wall damage, and disturbances of innate and acquired immune responses with autoantibody production are prominent features. Systemic sclerosis has specific subsets with different autoantibodies, and differences in the affected skin areas. The suspicion of systemic sclerosis and establishing the diagnosis will be facilitated by the criteria created by EULAR/ACR experts. The treatment of this autoimmune disease remains a challenge for clinicians and new therapeutic options are constantly sought. The occurrence of various symptoms and the involvement of many organs and systems make systemic sclerosis a multidisciplinary disease and require a holistic approach. The present article summarizes different clinical features of systemic sclerosis and the profile of autoantibodies and discusses recent rules and future perspectives in disease management.

Keywords: fibrosis; new therapies; systemic sclerosis.

Fig. 1

Images of SSc hands (comparison of radiograph and photograph).

Fig. 2

Images of nailfold capillaroscopy from normal image (A), early (B), active (B) to late stage (D) with giant capillaries and avascular areas.

Fig. 3

Skin biopsy with atrophic areas, increased collagen deposition with diminution of the spaces between normal collagen bundles.