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