Scleroderma in South Australia: epidemiological observations of possible pathogenic significance
Version of Record online: 21 DEC 2001
Internal Medicine Journal
Volume 31, Issue 4, pages 220–229, May/June 2001
How to Cite
Roberts-Thomson, P. J., Jones, M., Hakendorf, P., Kencana Dharmapatni, A. A. S. S., Walker, J. G., Macfarlane, J. G., Smith, M. D. and Ahern, M. J. (2001), Scleroderma in South Australia: epidemiological observations of possible pathogenic significance. Internal Medicine Journal, 31: 220–229. doi: 10.1046/j.1445-5994.2001.00048.x
- Issue online: 21 DEC 2001
- Version of Record online: 21 DEC 2001
- stochastic illness;
- systemic sclerosis
Background: Scleroderma is an autoimmune disorder of unknown cause. Previous epidemiological studies have suggested some regional clustering and associations with occupations involving exposure to silica dusts and hydrocarbons.
Aims: To determine: (i) prevalence and incidence of scleroderma in South Australia (SA), a state with a stable population living in well-defined urban, rural and industrial regions, (ii) hospital separation rates, (iii) cumulative survival rates, (iv) gender and disease subclassification, (v) geographical residency and occupations, (vi) familial associations and (vii) age at onset versus age-specific incidence rate.
Methods: Creation of the South Australian Scleroderma Register (SASR) to identify demographics and clinical and serological features of all scleroderma patients resident in SA.
Results: Scleroderma occurs in South Australia with a point prevalence of 23 per 105 and an incidence of approximately 1/15th of this. However, this prevalence is high compared with other regional world studies. Scleroderma is predominantly a female disease, with most patients having the limited form of scleroderma characterized by the presence of the anticentromere antibody and a mean survival of 27.6 years. In contrast, diffuse scleroderma is a less benign disease occurring at an early age of onset and has a mean survival of 9.6 years. Scleroderma occurs throughout SA without regional localization. Weak associations are seen in males, but not females, with occupations involving possible environmental exposure to silica or hydrocarbons. Scleroderma rarely occurs in families.
Conclusion: No strong genetic or environmental influences were found to account for the relatively common occurrence of scleroderma in SA. The age at onset versus age-specific incidence curve suggests that scleroderma can be considered as a stochastic illness involving a number of random events occurring in a predisposed population. These random events may involve mutations of pivotal somatic genes. (Intern Med J 2001; 31: 220–229)