title: Scleroderma in South Australia: epidemiological observations of possible pathogenic significance.
abstract: 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.
  
  
  OBJECTIVE
  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 10(5) 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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Immunology, Allergy and Arthritis, Flinders Medical Centre, Adelaide, South Australia. peter.roberts-thomson@flinders.edu.au
authors.forename: P J
authors.initials: PJ
authors.lastname: Roberts-Thomson
authors.forename: M
authors.initials: M
authors.lastname: Jones
authors.forename: P
authors.initials: P
authors.lastname: Hakendorf
authors.forename: A A
authors.initials: AA
authors.lastname: Kencana Dharmapatni
authors.forename: J G
authors.initials: JG
authors.lastname: Walker
authors.forename: J G
authors.initials: JG
authors.lastname: MacFarlane
authors.forename: M D
authors.initials: MD
authors.lastname: Smith
authors.forename: M J
authors.initials: MJ
authors.lastname: Ahern
mesh_terms: D017668:Age of Onset; D005260:Female; D006801:Humans; D008297:Male; D009790:Occupations / Q000706:statistics & numerical data; D010351:Patient Discharge / Q000706:statistics & numerical data; D012042:Registries; D012111:Residence Characteristics / Q000706:statistics & numerical data; D012595:Scleroderma, Systemic / Q000453:epidemiology* / Q000473:pathology; D013021:South Australia / Q000453:epidemiology; D015996:Survival Rate