Systemic sclerosis (SSc), a form of scleroderma, is a chronic, rare, debilitating systemic autoimmune disease affecting approximately 200,000 people in the U.S., EU and Japan.1
SSc affects the skin and internal organs and is driven by inflammation and fibrosis (scarring of tissue) leading to severe damage and failure of multiple organs, including the skin, joints, tendons, gastrointestinal tract, lungs, heart, blood vessels and kidneys.2
The symptoms of SSc vary depending upon which organs are affected and how badly they are affected. One type of SSc, diffuse cutaneous systemic sclerosis (dcSSc), has more widespread skin thickening, which may develop quickly and affect the upper extremities (for example, hand, wrist, arm) and trunk. The skin may become swollen, feel tight and itchy, and have pigment changes. Other symptoms of SSc include tender and swollen joints, shortness of breath, difficulty exercising, heartburn, trouble swallowing food, renal failure, Raynaud's phenomenon, high blood pressure and finger ulcers.2
The prognosis varies significantly among people affected and depends on what organs are affected by the disease. Unfortunately, SSc has the highest mortality rate among the systemic autoimmune diseases.4
There is no cure for systemic sclerosis, and current treatments address the clinical manifestations of the disease, not the underlying mechanisms that drive inflammation and fibrosis.5
Individuals living with scleroderma talk about the disease, symptoms, and how it affects their daily lives.
1. Health Advances, LLC Analysis
2. Sierra-Sepulveda A, Esquinca-Gonzalez A, Benavides-Suarez SA, Sordo-Lima DE, Caballero-Islas AE, Cabral-Castaneda AR, et al. Systemic Sclerosis Pathogenesis and Emerging Therapies, beyond the Fibroblast. Biomed Res Int. 2019;2019:4569826
3. “Scleroderma.” National Institute of Arthritis and Musculoskeletal and Skin Diseases, U.S. Department of Health and Human Services, 27 Aug. 2018, niams.nih.gov/health-topics/scleroderma/advanced#tab-risk 3. Solomon, J. J., et al. “Scleroderma Lung Disease.” European Respiratory Review, vol. 22, no. 127, 2013, pp. 6–19., doi:10.1183/09059180.00005512
4. Elhai M, Meune C, Avouac J, Kahan A, Allanore Y. Trends in mortality in patients with systemic sclerosis over 40 years: a systematic review and meta-analysis of cohort studies. Rheumatology 2012;51(6):1017e26
5. Scleroderma.” National Institute of Arthritis and Musculoskeletal and Skin Diseases, U.S. Department of Health and Human Services, 20 May 2020, www.niams.nih.gov/health-topics/scleroderma/advanced#tab-risk.