Systemic sclerosis prevalence

The cause of systemic sclerosis is unknown. Ninety-five percent of patients have identifiable autoantibodies. There are two major subsets of the disease, fimited cutaneous and diffuse systemic sclerosis. Patients with limited cutaneous involvement often have the CREST syndrome (calcinosis, Raynaud s, esophageal dysmotility, sclerodactyly, and felangiectasias), whereas patients with diffuse systemic sclerosis have a more aggressive disease with renal, cardiac, or pulmonary involvement. The prevalence of the disease is estimated to be between 138 and 286 cases per 1 million population. The wide range may be due to differences in diagnostic criteria, regional variation, or sample sizes used to estimate the prevalence. 

Mayers MD, Lacey JV Jr, Beebe-Dimmer J, et al. Prevalence, incidence, survival, and disease characteristics of systemic sclerosis in a large US population. Athritis Rheum 2003 48 2246-2255. 

Scleroderma (systemic sclerosis) is a disease of unknown etiology characterized by abnormally increased collagen deposition in the skin and visceral organs, alterations in the microvasculature, and numerous cellular and humoral immune abnormalities. A recent study in the United States estimated the disease prevalence to be approximately 25 per 100 000 (Mayes et al., 2003). Besides a racial... 

Barnes J, Mayes MD. Epidemiology of systemic sclerosis incidence, prevalence, survival, risk factors, malignancy, and environmental triggers. Curr Opin Rheumatol. 2012 24(2) 165—170. 

Arnett FC, Howard RF, Tan F, et al. Increased prevalence of systemic sclerosis in a Native American tribe in Oklahoma. Association with an Amerindian HLA hap-lotype. Arthritis Rheum 1996 39(8) 1362-1370. 

Launay D, Mouthon L, Hachulla E, et al. Prevalence and characteristics of moderate to severe pulmonary hypertension in systemic sclerosis with and without interstitial lung disease. J Rheumatol 2007 34 1005-1011. 

Systemic sclerosis (SSc), also named scleroderma, is a systemic disease characterised by thickening of the skin due to an accumulation of connective tissue and by the involvement of various organs such as the lungs, gastrointestinal tract, heart and kidneys. The annual incidence has been estimated to be 19 cases per million population, and the reported prevalence of SSc is between 19 and 75 per 100,000 persons. 

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