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


Over the past years, there have been several studies regarding incidence and prevalence of SSc. The reported studies continue to show variation by geographic region. Incidence rates and prevalence estimates are fairly similar for Europe, the United States, Australia, and Argentina suggesting a prevalence of 150–300 cases per million with a lower prevalence noted in Scandinavia, Japan, the UK, Taiwan, and India.

Although survival rates have improved over time, pulmonary fibrosis and pulmonary arterial hypertension continue to be the leading causes of SSc-related deaths. In all studies, SSc is more common in women than in men and the cause of this discrepancy remains speculative. African-American (US study) and older age onset (multiple countries) patients have more severe disease.

This past year has seen new evidence for the role of genetic factors in SSc occurrence and disease severity, particularly scleroderma renal crisis. The association of autoantibodies with particular disease manifestation has been further validated.

Although there is evidence to suggest an association between environmental exposures and SSc, such exposures only contribute to a small proportion of all cases. Cigarette smoking was not found to increase the risk of developing SSc but appears to contribute to disease severity. It is likely that there is an increased risk of cancer among SSc patients, but reports vary about the magnitude of this risk and the type of malignancy.


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