Systemic sclerosis & Raynaud's
Long-Term Epidemiology of Systemic Sclerosis in Western Australia: A Population-Level Linked Data Study
Authors' conclusion
The burden of SSc in WA exceeds global estimates and its high prevalence, high SMR, and number of deaths due to SSc as a primary cause suggest a large unmet therapeutic need.
Study context
- Population
- Patients with an incident (first-ever) hospitalization with systemic sclerosis (SSc) in Western Australia (WA), 1985–2013, followed until end of 2014 (WA Rheumatic Disease Epidemiological Registry)mean age 58.6 years, 77.8% female
- Participants
- 877 patients
- Exposure
- Male vs Female
- Outcome
- Prevalence, incidence and mortality of SSc (IR, point prevalence, SMR, survival)
Methodological detailspopulation-level linked administrative health data, stratified by sex
- Population-level linked administrative health data
- Stratified by sex
Main finding
As reported in the abstract
Prevalence, incidence and mortality of SSc
- Age-standardized incidence rate (IR) of SSc, per 100,000 person-years (range)
- 0.44 to 3.26
- Point prevalence, average per 100,000 population
- 37.93
- Deaths during study period
- 452 (51.5%)
- Standardized mortality ratio (SMR)
- 4.17 (95% CI 3.81-4.58)
- 5-year survival
- 67%
- 10-year survival
- 52.4%
Observational data: associations, not effects.
Further findings
Crude mortality by sex
P < 0.001
Factors independently associated with death (Cox regression)
| HR (95% CI) | ||
|---|---|---|
| Age | 1.05 (1.04–1.05) | |
| HF | 1.88 (1.36–2.60) | |
| Kidney disease | 1.71 (1.13–2.58) | |
| Cancer | 1.88 (1.30–2.74) | |
| 0.20.5125Hazard ratio (log scale) |
| Outcome | HR (95% CI) | |
|---|---|---|
| Factors independently associated with death (Cox regression)Malevs Female | 1.56 (1.27–1.92) | |
| 0.512Hazard ratio (log scale) |
Main causes of death
- SSc
- n = 128, 28.3%
- Solid organ malignancy
- n = 65, 14.4%
- Ischemic heart disease
- n = 47; 10.4%
Original publication
Long-Term Epidemiology of Systemic Sclerosis in Western Australia: A Population-Level Linked Data Study. J Rheumatol. Published 1 October 2025. doi:10.
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