Incidence and Prevalence of Systemic Sclerosis: A Systematic Literature Review
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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