[Modern perspective on the development conditions and diagnosis of systemic sclerosis]

Systemic sclerosis (SSc) is a multisystem disorder of the connective tissue characterized by a great deal of heterogeneity. This variability is a result of a combination of vascular damage inflammation and fibrosis leading to internal organ complications. The pathogenesis of disorder is still unknown. A large part is given to genetic, epigenetic and environmental factors. There is ongoing research into the new cells and mediators involved in the complicated development of disease. The overlap of vascular, autoimmune/ inflammatory alternations and fibrosis causes the multidirectional and unpredictable course of disease. Vascular complications may dominate in one group, whereas fibrosis in others. In addition, the disorder is characterized by individual variability; therefore although certain prognostic markers do exist, the course of disorder in largely unpredictable. This complexity of SSc, makes it difficult in terms of diagnosis and activity assessment, especially in early stages. Over a number of years, further classification criteria, which were more sensitive and more specific, were developed. The 2013 European League Against Rheumatism/American College of Rheumatology - EULAR/ACR classification criteria for SSc, were revised to include diagnosis in early stages and particularly in limited SSc. Unfortunately, there still exist certain groups of population which do not fulfil these criteria but develop the disorder; therefore, the search for new diagnostic methods which could elevate SSc diagnosis continues.