Abstract
Systemic sclerosis is a severe, potentially life threatening autoimmune disease involving the skin and visceral organs, including the gastrointestinal tract, lungs, heart and kidneys, with an estimated 10-year survival rate of approximately 50% and an annual incidence of 0.6–19.0 cases per million people. The chronicity and the peak of disease onset between 30 and 55 years underline the socioeconomic impact of systemic sclerosis. Although significant advances have been made in providing symptomatic organ-specific therapy and a multitude of drugs with immunosuppressive and antifibrotic properties have been tried, for most drugs, convincing controlled clinical studies are missing. Improvement of diagnostic measures and novel drugs may provide the basis for more successful therapeutic approaches in the future.
Notes
ACA: Anticentromere antibody; ACR: American College of Rheumatology; SSc: Systemic sclerosis.