Systemic sclerosis - multidisciplinary disease: clinical features and treatment.

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.

Potential occupational and environmental factors in SSc onset.

INTRODUCTION: Introduction and objective. Systemic sclerosis [SSc; scleroderma] is a rare, connective tissue disorder affecting all organs and systems. The primary feature of this disease is a chronic, progressive fibrosis due to excessive production of collagen and other components. There are two subsets of systemic sclerosis: 1) diffused SSc (dSSc), 2) limited SSc (lSSc) and 3) scleroderma without sclerosis (SSSC). The presented review is an attempt to summarize recent data regarding environmental and occupational factors in SSc onset. STATE OF KNOWLEDGE: There are many factors to be taken under consideration with SSc onset, although a strong correlation has been established for only a few. The most distinct factors are: crystalline silica and organic solvents (such as white spirit, aromatic, aliphatic-chain, chlorinated solvents, ketones, welding fumes). For other factors, which include abstestos, air pollution, other chemicals, silicone breast implants, tobacco smoking, drug reactions, diet influence and exposure to heavy metals, the jury is still out, and their position in SSc onset needs further studies. CONCLUSIONS: Conclusions. Although the pathogenesis of scleroderma remains unclear, there is a marked correlation between the onset of SSc and certain environmental or occupational factors.