Systemic Sclerosis (SSc)
Epidemiological studies have underscored the incidence of SSc in the sixth, seventh and eighth decades of life. The pathogenesis of systemic sclerosis (SSc) is characterised by immune and endothelial activation, vascular dysfunction and overproduction of extracellular matrix. The late onset is clinically and immunologically different from the early onset. There are two subsets of SSc, diffuse cutaneous SSc (dcSSc) which is associated with progressive fibrosis of skin and internal organs and the other limited cutaneous.
KeywordsSystemic sclerosis Late onset Early onset Diffuse cutaneous Diffuse cutaneous CREST syndrome
- 2.Chatterjea S. Systemic scleroderma. http://www.clevelandclinicmeded.com/medicalpubs/diseasemanagement/rheumatology/systemicsclerosis/Default.htm acceessed 27 April 2015.
- 3.Mayes MD, Lacey JU, Beebee-Dimmer J, Gillepsie BW, Cooper B, Liang TJ, et al. Prevalence incidence survival and disease characteristics of systemic sclerosis in a large US population. Arthritis Rheum. 2003;48:246–55.Google Scholar
- 6.Denton CP. Pathogenesis of systemic sclerosis (scleroderma). UpToDate. http://www.update.com/contents/pathogensis-of-systemic-sclerosis-scleroderma. Accessed 27April 2015.
- 10.Hugle T, Schuetz P, Daikeler T, Tyndall A, Matucci-Cerinic M, Walker UA, et al. Late-onset systemic sclerosis – a systematic survey of the EULAR scleroderma trials and research group database. Rheumatology. (2010). https://doi.org/10.1093/rheumatology/keq321.
- 12.Systemic sclerosis in old age. Br Med J. 1979;2(6201):1313–4.Google Scholar
- 18.University of Maryland Medical Center. Scleroderma. http://umm.edu/health/medical/reports/articles/scleroderma accessed on 25 September, 2015.
- 19.Hummers LK, Wriley F. Scleroderma treatment options. http://www.hopkinsscleroderma.org/patients/scleroderma-treatment-options/ accessed on 25 September 2015.