Abstract
Systemic sclerosis (SSc) is a multisystem autoimmune rheumatic disease which forms part of the scleroderma spectrum of disorders. These diverse conditions are listed in Table 1. There are common features to these disorders and some clinical overlap between them but they can be separated into three main categories, those with skin sclerosis but no vascular or internal organ involvement such as morphea, conditions with vascular manifestations only, including primary Raynaud’s phenomenon, and the different subsets of systemic sclerosis, which form the main focus of this article. The frequency of the disorders has proven difficult to ascertain but North American studies suggest a prevalence of Raynaud’s phenomenon of around 10% of the adult female population (6% overall), and an annual prevalence of SSc of 15 cases per 100,000. Data from the UK has previously estimated a much lower prevalence but a comprehensive local study has led to estimates being revised to levels similar to the North American figures [28].
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