A systematic review of the epidemiology, disease characteristics and management of systemic sclerosis in Australian adults

Abstract

Objective: Australia has one of the highest prevalence rates of systemic sclerosis (SSc) worldwide. In order to highlight management deficiencies and key areas for further research, it is essential to understand its local epi- demiological patterns, natural history, prognosis and mortality trends over time. Methods: To identify Australian SSc-specific information through a systematic review focusing on areas of epi- demiology, disease characteristics, treatment, functional ability, disease burden and health-related quality of life (HRQoL). Results: MEDLINE, EMBASE and the Cochrane Library were searched on 14 September, 2016. All original full text articles on SSc in Australia were included. Of the 54 articles included in this review, the majority of studies recruited from South Australia, Victoria and New South Wales. The prevalence of SSc in Australia is increasing and is similar among the general population and the Aboriginal population. Despite improvements in care over the last three decades, morbidity and mortality remain high, with an overall standardized mortality ratio of 3.4 and a 10- year survival of 84% in a newly diagnosed patient. Cardiorespiratory manifestations are the leading cause of SSc- related death. Malignancy is the leading cause of non-SSc-related death. The role of autoantibodies in predicting disease subtype, visceral involvement and their use as a prognostic marker is becoming increasingly recognized. Conclusion: Information on SSc in Australia, particularly unmet healthcare needs, HRQoL and economic bur- den, is limited. As a heterogenous condition, SSc requires a multi-disciplinary approach to care. Research aimed at quantifying HRQoL and burden of disease in Australia is essential for advocacy and resource allocation.