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.

Vascular

Raynaud

Systemic sclerosis (SSc) is a rare, auto immunological, connective tissue disease characterized by vasomotor disturbances, fibrosis and further atrophy of the skin and internal organs. There are many factors leading to immunological and vascular changes. One of the most prominent vascular symptoms is Raynaud's phenomenon, which may be caused by different factors such as cold or excessive stress. This case report presents a 37-year old Caucasian woman with SSc, with severely impaired microcirculation of the hands and necrosis of the distal phalanges due to several hours of exposure to the cold. The patient required intensification of intravenous rheological treatment with alprostadil and oral usage of sildenafil, which had a positive effect on the improvement of the impaired microcirculation. Like many others, this case demonstrates that IV. rheological treatment may be effective in restoring microcirculation in such cases among SSc patients.