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.

This study substantially increases the otherwise small list of documented instances of familial systemic sclerosis. More importantly, it quantifies the risk for the first time, ranking it as the disease's most powerful determinant identified to date.

Male systemic sclerosis displays socioeconomic dependence. Silica is a disease determinant in male systemic sclerosis, with disease features including a long latency and clinical characteristics indistinguishable from idiopathic disease. Cross-sectional 'current' occupational data underestimate cumulative occupational silica exposure.

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No association was found between augmentation mammoplasty exposure and various connective tissue diseases and/or their related features. However, axillary adenopathy and low titre ANA were detected more frequently in the exposed cohort. Women with axillary adenopathy were more likely to have breast capsular contracture and report digital vasospasm post-dating surgery. Given comparable frequencies of higher titre ANA of both cohorts, the finding of elevations of low titre ANA is of dubious clinical significance.