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Early- versus late-onset systemic sclerosis: differences in clinical presentation and outcome in 1037 patients. | LitMetric

Early- versus late-onset systemic sclerosis: differences in clinical presentation and outcome in 1037 patients.

Medicine (Baltimore)

From Department of Autoimmune Diseases (MAA, GE) and Department of Epidemiology Medicine (CV), Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Catalonia; Department of Internal Medicine (CPS, VF), Hospital Vall d´Hebron, Barcelona; Department of Internal Medicine (LT), Hospital Universitario Central de Asturias, Oviedo, Asturias; Department of Internal Medicine (MVE), Hospital de Cruces, Barakaldo, Vizcaya; Department of Internal Medicine (LS), Hospital Universitario Miguel Servet, Zaragoza; Department of Collagenosis and Pulmonary Hypertension (MJC), Hospital Universitario Virgen del Rocio, Sevilla; Department of Internal Medicine (JLC), Hospital Universitario San Cecilio, Granada; Department of Internal Medicine (MTC), Hospital Regional Universitario Carlos Haya, Málaga; Department of Internal Medicine (CT), Corporacíon Sanitaria Universitaria Parc Taulí, Sabadell, Barcelona; Department of Internal Medicine (JJR), Hospital Universitario La Paz, Madrid; Department of Internal Medicine (MF), Complejo Hospitalario Universitario de Vigo, Vigo, Pontevedra; Department of Internal Medicine (JAV), Hospital Universitario Virgen de las Nieves, Granada, Spain.

Published: March 2014

AI Article Synopsis

Article Abstract

Peak age at onset of systemic sclerosis (SSc) is between 20 and 50 years, although SSc is also described in both young and elderly patients. We conducted the present study to determine if age at disease onset modulates the clinical characteristics and outcome of SSc patients. The Spanish Scleroderma Study Group recruited 1037 patients with a mean follow-up of 5.2 ± 6.8 years. Based on the mean ± 1 standard deviation (SD) of age at disease onset (45 ± 15 yr) of the whole series, patients were classified into 3 groups: age ≤ 30 years (early onset), age between 31 and 59 years (standard onset), and age ≥ 60 years (late onset). We compared initial and cumulative manifestations, immunologic features, and death rates. The early-onset group included 195 patients; standard-onset group, 651; and late-onset, 191 patients. The early-onset group had a higher prevalence of esophageal involvement (72% in early-onset compared with 67% in standard-onset and 56% in late-onset; p = 0.004), and myositis (11%, 7.2%, and 2.9%, respectively; p = 0.009), but a lower prevalence of centromere antibodies (33%, 46%, and 47%, respectively; p = 0.007). In contrast, late-onset SSc was characterized by a lower prevalence of digital ulcers (54%, 41%, and 34%, respectively; p < 0.001) but higher rates of heart conduction system abnormalities (9%, 13%, and 21%, respectively; p = 0.004). Pulmonary hypertension was found in 25% of elderly patients and in 12% of the youngest patients (p = 0.010). After correction for the population effects of age and sex, standardized mortality ratio was shown to be higher in younger patients. The results of the present study confirm that age at disease onset is associated with differences in clinical presentation and outcome in SSc patients.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4616306PMC
http://dx.doi.org/10.1097/MD.0000000000000018DOI Listing

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