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A systematic review of the epidemiology, disease characteristics and management of systemic sclerosis in Australian adults
Author(s) -
Morrisroe Kathleen,
Stevens Wendy,
Proudman Susanna,
Nikpour Mandana
Publication year - 2017
Publication title -
international journal of rheumatic diseases
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.795
H-Index - 41
eISSN - 1756-185X
pISSN - 1756-1841
DOI - 10.1111/1756-185x.13203
Subject(s) - medicine , epidemiology , disease , medline , population , cochrane library , cause of death , intensive care medicine , mortality rate , gerontology , environmental health , meta analysis , law , political science
Objective Australia has one of the highest prevalence rates of systemic sclerosis ( SS c) worldwide. In order to highlight management deficiencies and key areas for further research, it is essential to understand its local epidemiological patterns, natural history, prognosis and mortality trends over time. Methods To identify Australian SS c‐specific information through a systematic review focusing on areas of epidemiology, disease characteristics, treatment, functional ability, disease burden and health‐related quality of life ( HRQ oL). Results MEDLINE , EMBASE and the Cochrane Library were searched on 14 September, 2016. All original full text articles on SS c 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 SS c 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 SS c‐related death. Malignancy is the leading cause of non‐ SS c‐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 SS c in Australia, particularly unmet healthcare needs, HRQ oL and economic burden, is limited. As a heterogenous condition, SS c requires a multi‐disciplinary approach to care. Research aimed at quantifying HRQ oL and burden of disease in Australia is essential for advocacy and resource allocation.