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.

Malignancy

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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Next Section

Abstract and Introduction
Incidence and Prevalence of Systemic Sclerosis
Survival
Environmental Triggers
Risk Factors
Malignancy
Conclusion
References

Table 1. Incidence and prevalence in past decades
Publication	Region	Study period	Incidence per million	Prevalence per million
USA
Steen et al. [4]	Pennsylvania	1963–1972, 1973–1982, 1963–1982	13.9 (entire period)
Maricq et al. [5]	South Carolina	1989	–	286
Mayes et al. [6]	Michigan	1989–1991	21	276
Arnett et al. [7]	Oklahoma	1996		658.6
Bernatsky et al. [8]	Quebec	2003	–	443
Australia
Englert et al. [9]	Sydney	1974–1988	12	45.2 (1975) 86.2 (1988)
Chandran et al. [10]	South Australia	1987–1993	–	208
Roberts-Thompson et al. [11]	South Australia	1993	15.1	200
		1999	22.8	233
Roberts-Thompson et al. [12]	South Australia	1993–2002	20.4	232.2
Japan
Tamaki et al. [13]	Tokyo	1987	7.2	38
UK and Europe
Silman et al. [14]	England (West Midlands)	1986	3.7	31
Allock et al. [15]	England (Newcastle)	2000	–	88
Geirsson et al. [16]	Iceland	1975–1990	3.8	71
Kaipiainen-Seppanen	Finland	1990	3.7
and Aho [17]
Le Guern et al. [18]	France (Seine-St-Denis	2001	–	158
Alamanos et al. [19]	Greece (Northwest)	1981–2002	11	154
Arias-Nunez et al. [20]	Spain (Northwest)	1988–2006	23	277

Table 2. Incidence and prevalence: updates in last year
Publication	Region	Study period	Incidence per million	Prevalence per million
Monaco et al. [21•]	Northeast Italy	1991–2007	43	341
Rosa et al. [22•]	Buenos Aires	1999–2004	21.2	296
Kuo et al. [23•]	Taiwan	2002–2007	10.9	56
Minz et al. [24•]	North India	2006–2007	–	120

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