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.

Conclusion

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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Acknowledgements
None.

Conflicts of interest
The authors have no conflicts of interest.

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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