Late-onset systemic sclerosis--a systematic survey of the EULAR scleroderma trials and research group database.
OBJECTIVE
The clinical course of SSc depends on subtype, organ involvement and age. Few data are reported on patients suffering from late-onset SSc.


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.


CONCLUSIONS
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.
Hügle
Thomas
T
Musculoskeletal Research Group, Institute of Cellular Medicine, The Medical School, Framlington Place, Newcastle University, Newcastle upon Tyne NE2 4HH, UK. t.huegle@ncl.ac.uk
Schuetz
Philipp
P
Daikeler
Thomas
T
Tyndall
Alan
A
Matucci-Cerinic
Marco
M
Walker
Ulrich A
UA
van Laar
Jacob M
JM
D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D002986:Clinical Trials as Topic; D016208:Databases, Factual; D004812:Epidemiologic Methods; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D011446:Prospective Studies; D012044:Regression Analysis; D012594:Scleroderma, Localized / Q000453:epidemiology / Q000503:physiopathology*; D012595:Scleroderma, Systemic / Q000453:epidemiology / Q000503:physiopathology*; D012720:Severity of Illness Index; D013997:Time Factors