Incidence and Prevalence of Systemic Sclerosis: A Systematic Literature Review

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Objectives

To determine the incidence and prevalence of systemic sclerosis (SSc) in adults, its epidemiological tendencies over time, and its possible key determinants.

Methods

We performed a systematic literature review using the keywords “systemic sclerosis,” “incidence,” “prevalence,” and “epidemiology.”

Results

We found 32 articles published from 1969 to 2006 in which the prevalence of SSc ranged from 7/million to 489/million and its incidence from 0.6/million/y to 122/million/y. There were many geographical variations: SSc prevalence was higher in the USA (276/million in 1990) and Australia (233/million in 1999) than in Japan and Europe, where a north–south gradient was also observed (France: 158/million in 2001 and England: 88/million in 2000). In some regions (Ontario, Rome, near London’s airports) there was an unusually high number of SSc cases (3, 5, or 1000 times greater than expected), suggesting spatiotemporal clustering, although no key determinants could be identified. Furthermore, there seemed to be a trend toward an increase in the incidence of SSc over time, but this tendency is uncertain due to lack of uniformity in study methods and designs. We also found that susceptibility to the disease differed according to sex, age, and race.

Conclusion

Uniform clinico-epidemiological studies with standard diagnostic and classification criteria are needed to refine the epidemiological features of SSc. Homogeneous study methods with exhaustive case ascertainment as seen in a “capture-recapture” analysis will also be necessary to obtain reliable data.

Section snippets

Methods

A systematic analysis of the literature was performed. The inclusion criteria for this research were original articles in English or French concerning the prevalence and incidence of SSc in adults (reports concerning the disease in childhood or localized forms of scleroderma (morphea) were excluded). In these reports, the incidence and prevalence of SSc had to be directly mentioned or easily calculated from the available data (the total number of SSc cases at the endpoint of the study period

Results

SSc has been described in patients all over the world. We collected studies (Table 1, Table 2, Table 3) reporting the prevalence and incidence of SSc over 55 years (from 1947 to 2002) in nearly all continents (except Africa). There were wide variations in the results.

Discussion

SSc is characterized by its low-frequency, insidious onset and variable presentation and the confusion in its classification, making it difficult to study its epidemiological features. Surveys concerning the worldwide prevalence and incidence of SSc are moreover quite heterogeneous and therefore difficult to compare.

The first problem is the lack of uniformity in the diagnostic criteria. The 1980 SSc classification criteria in (19) (originally developed to distinguish SSc from other connective

Acknowledgments

This review was conducted with the support of Prof. Maxime Dougados and his colleagues of the Department of Rheumatology, Cochin Teaching Hospital, Paris, France and the COFER.

We also thank Dr. Sylviane Muller and Dr. Hélène Dumortier for critical reading of the manuscript.

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