Summary
Systemic sclerosis (SSc) is an autoimmune systemic disease characterized by small vessel involvement that leads to tissue ischemia and fibroblast stimulation resulting in accumulation of collagen (fibrosis) in the skin and internal organs. The peak incidence of the disease is found between the third and fifth decade of life. The male to female ratio is 5:1. Annual incidence is 14.1 cases per million. Prevalence ranges from 19 to 75 cases/ 100 000 people. No well-defined treatment has been found. However several types of treatment exist and can be classified as such A) systemic therapies and B) organ - specific therapies. Systemic therapies are subdivided into vascular therapies, immunomodulating therapies and antifibrotic therapies. Organ-specific therapies are subdivided into therapy of pulmonary interstitial fibrosis, therapy of pulmonary hypertension, therapy of SSc renal crisis. Most of the above-mentioned therapies have been tested in open clinical trials. Systemic sclerosis is more common in coal and gold miners and miners exposed to vinyl-chloride, epoxyresins and aromatic hydrocarbons. However, these factors do not explain the spontaneously developed disease. Recently, CD34 stem cells of child origin were detected in women with SSc more commonly than in normal women. Furthermore, the SSc women display histocompatibility in most of the HLA loci with their children. *Author: P.G. Vlachoyiannopoulos, MD (November 2001)*.
