Late-onset systemic sclerosis—a systematic survey of the EULAR scleroderma trials and research group database

Rheumatology, 10/04/2010

Hügle T et al. – Late-onset SSc shows a distinct clinical presentation and outcome. Patients with late-onset SSc suffer more frequently from the limited subtype and PH, but fewer patients have digital ulcers. PH may in part be determined by underlying cardiovascular disease.


  • analysed data from 8554 patients prospectively followed in the EULAR Scleroderma Trials and Research (EUSTAR) group database
  • Late-onset SSc defined as onset of non-RP disease features at or beyond 75 years of age
  • Disease characteristics, clinical features, disease course and mortality evaluated


  • Total of 123 patients with SSc onset at or beyond 75 years of age identified
  • Compared with patients <75 years had more frequently limited than diffuse SSc and higher prevalence of anti-centromere autoantibodies
  • Fewer old patients had digital ulcers
  • Modified Rodnan’s skin score, prevalence of lung fibrosis and renal crisis did not differ significantly between groups
  • Pulmonary hypertension (PH) measured by echocardiography more prevalent in late-onset group, as well as arterial hypertension and diastolic dysfunction
  • Late-onset SSc remained positive predictor for PH in multivariate analyses
  • Mortality due to SSc higher in late-onset group
  • Survival time from diagnosis longer compared with younger patients

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