Systemic Sclerosis Prevalence and Mortality in Sydney, 1974-88

BACKGROUND: Systemic sclerosis prevalence and mortality estimates have demonstrated wide variability. The sole Australian study published to date demonstrated high prevalence rates when compared to overseas estimates. The prevalence and mortality findings reported in this paper derive from a larger study which addressed the distribution and determinants of systemic sclerosis within Sydney. AIMS: To determine systemic sclerosis prevalence and mortality rates within Sydney over 15 years, 1974-88. METHODS: Cases were ascertained from multiple sources including death certificates, hospitals, physicians, vascular surgeons' and dermatologists' private practices, a systemic sclerosis self-help group and private medical laboratories. RESULTS: Overall, 715 cases were identified. Females comprised 77% (95% CI: 74-80) of cases. Disease of the limited subtype accounted for 79% (95% CI: 76-82) of all systemic sclerosis, being relatively more frequent in living than deceased cases, and in females than males. Crude prevalence estimates appeared to rise between 1975 (4.52/100,000 95% CI:3.75-5.29/100,000) and 1988 (8.62/100,000 95% CI:7.64-9.60/100,000) as did estimates of diffuse disease. However, diffuse disease prevalence, when expressed as a proportion of total disease prevalence, showed no significant temporal change. Although crude mortality rates also showed apparent temporal increases (0.24/100,000 in 1975 to 0.80/100,000 in 1988) standardised mortality rates showed less convincing trends (0.41/100,000 in 1976 and 0.40/100,000 in 1988). Death certificate-derived mortality rates provided relatively large underestimates of total mortality. However, these underestimates were relatively constant over time. CONCLUSIONS: This study has demonstrated systemic sclerosis prevalence and mortality rates comparable to overseas estimates, consistently higher prevalence and mortality rates in females than males, proportionally higher rates of diffuse disease in males than females and in deceased cases than living cases, a diffuse: limited disease ratio apparently stable over time, apparently increasing temporal prevalence and mortality rates and, by implication, rising incidence rates. The observed temporal rise in diffuse disease prevalence and the absence of a convincing fall in diffuse disease mortality suggests a rising temporal incidence rate of diffuse disease. Standardised mortality rates demonstrated less consistent trends than did crude mortality rates and failed to demonstrate convincing declines in mortality subsequent to the introduction of ACE inhibitors for management of systemic sclerosis renal disease. Death certificate-derived systemic sclerosis mortality rates considerably and consistently underestimated systemic sclerosis-all cause mortality