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

Lookup NU author(s): Dr Thomas Huegle, Professor Jaap Van Laar


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Methods. We analysed data from 8554 patients prospectively followed in the EULAR Scleroderma Trials and Research (EUSTAR) group database. Late-onset SSc was defined as onset of non-RP disease features at or beyond 75 years of age. Disease characteristics, clinical features, disease course and mortality were evaluated. Results. A total of 123 patients with SSc onset at or beyond 75 years of age were identified. Compared with patients < 75 years they had more frequently limited than diffuse SSc and a higher prevalence of anti-centromere autoantibodies. Fewer old patients had digital ulcers. The modified Rodnan's skin score, the prevalence of lung fibrosis and renal crisis did not differ significantly between groups. Pulmonary hypertension (PH) measured by echocardiography was more prevalent in the late-onset group, as well as arterial hypertension and diastolic dysfunction. Late-onset SSc remained a positive predictor for PH in multivariate analyses. No significant difference of the two groups in skin score or diffusion capacity was observed during follow-up. Mortality due to SSc was higher in the late-onset group, but the survival time from diagnosis was longer compared with the younger patients. Conclusion. 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.

Publication metadata

Author(s): Hugle T, Schuetz P, Daikeler T, Tyndall A, Matucci-Cerinic M, Walker UA, van Laar JM, EUSTAR Members

Publication type: Article

Publication status: Published

Journal: Rheumatology

Year: 2011

Volume: 50

Issue: 1

Pages: 161-165

Print publication date: 30/09/2010

ISSN (print): 1462-0324

ISSN (electronic): 1462-0332

Publisher: Oxford University Press


DOI: 10.1093/rheumatology/keq321


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