Epidemiology of Systemic Sclerosis

Incidence, Prevalence, Survival, Risk Factors, Malignancy, and Environmental Triggers

Jammie Barnes; Maureen D. Mayes


Curr Opin Rheumatol. 2012;24(2):165-170. 

In This Article

Abstract and Introduction


Purpose of review To identify the recent data regarding prevalence, incidence, survival, and risk factors for systemic sclerosis (SSc) and to compare these data to previously published findings.
Recent findings SSc disease occurrence data are now available for Argentina, Taiwan, and India and continue to show wide variation across geographic regions. The survival rate is negatively impacted by older age of onset, male sex, scleroderma renal crisis, pulmonary fibrosis, pulmonary arterial hypertension, cancer, and antitopoisomerase and anti-U1 antibodies. It appears that silica exposure confers an increased risk for developing scleroderma, but this exposure accounts for a very small proportion of male patients. Smoking is not associated with increased SSc susceptibility. Malignancies are reported in scleroderma at an increased rate, but the magnitude of this risk and the type of cancer vary among reports.
Summary Prevalence and incidence of SSc appears to be greater in populations of European ancestry and lower in Asian groups. Exposure to silica dust appears to be an environmental trigger, but this only accounts for a small proportion of male cases. Evidence for increased risk of neoplasia is suggestive, but the magnitude of the risk and the types of malignancies vary among reports.


Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterized by small vessel vasculopathy, autoantibody production, and excessive collagen deposition in the skin and internal organs.[1] The classification of SSc is divided into two groups: limited and diffuse cutaneous disease.[2] The limited form is characterized by thickening of the skin confined to the areas distal to the elbows and knees, and is associated with less severe internal organ involvement. The diffuse form involves skin thickening proximal to the elbows and knees in addition to distal area involvement and is associated with more severe organ damage. This review will focus on the epidemiology of SSc and discuss the recently published articles related to this topic. The incidence and prevalence, survival, risk factors, malignancy, and putative environmental triggers will be reviewed.


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