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Title: Early- versus late-onset systemic sclerosis. Differences in clinical presentation and outcome in 1037 patients
Author: Alba Garibay, Marco Antonio
Velasco, César
Simeón Aznar, Carmen Pilar
Fonollosa Pla, Vicent
Trapiellla Martínez, Luis
Egurbide Arberas, María Victoria
Sáez Comet, Luis
Castillo Palma, María Jesús
Callejas Rubio, José Luis
Camps García, María Teresa
Tolosa Vilella, Carles
Ríos Blanco, Juan José
Freire, Mayka
Vargas Hitos, José A.
Espinosa Garriga, Gerard
RESCLE Registry
Keywords: Esclerodèrmia
Malalties autoimmunitàries
Scleroderma (Disease)
Autoimmune diseases
Issue Date: Mar-2014
Publisher: Lippincott, Williams & Wilkins. Wolters Kluwer Health
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.
Note: Versió postprint del document publicat a:
It is part of: Medicine, 2014, vol. 93, num. 2, p. 73-81
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ISSN: 0025-7974
Appears in Collections:Articles publicats en revistes (Medicina)
Articles publicats en revistes (IDIBAPS: Institut d'investigacions Biomèdiques August Pi i Sunyer)

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