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


In the past, several studies have studied the association of malignancy and scleroderma with sometimes conflicting findings.[36•]

In a recent Danish study from a national registry in the period 1997–2006, Olesen et al.[37] found 222 cases of cancer identified after the diagnosis of SSc. These authors reported an increased standardized incidence ratio for cancer of 1.5. Men had a higher incidence of cancer than women with a standardized incidence ratio of 2.2 (95% CI 1.7–2.8) for men and 1.3 (95% CI 1.1–1.6) for women. The most frequent malignancies reported were smoking-related and alcohol-related cancers.

Another cohort study in southwest England by Siau et al. [36•] studied malignancy in SSc patients and age-matched controls based on a regional healthcare database. They identified 15 malignancies out of 68 SSc patients. Although the total number of SSc cases was low, the authors calculated a relative risk of 3.15 (95% CI 1.77–5.20) in overall cancers and found the highest to be hematologic malignancies.


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