title: Early- versus late-onset systemic sclerosis: differences in clinical presentation and outcome in 1037 patients.
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.
authors.affiliation: 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.
authors.forename: Marco A
authors.initials: MA
authors.lastname: Alba
authors.forename: César
authors.initials: C
authors.lastname: Velasco
authors.forename: Carmen Pilar
authors.initials: CP
authors.lastname: Simeón
authors.forename: Vicent
authors.initials: V
authors.lastname: Fonollosa
authors.forename: Luis
authors.initials: L
authors.lastname: Trapiella
authors.forename: María Victoria
authors.initials: MV
authors.lastname: Egurbide
authors.forename: Luis
authors.initials: L
authors.lastname: Sáez
authors.forename: María Jesús
authors.initials: MJ
authors.lastname: Castillo
authors.forename: José Luis
authors.initials: JL
authors.lastname: Callejas
authors.forename: María Teresa
authors.initials: MT
authors.lastname: Camps
authors.forename: Carles
authors.initials: C
authors.lastname: Tolosa
authors.forename: Juan José
authors.initials: JJ
authors.lastname: Ríos
authors.forename: Mayka
authors.initials: M
authors.lastname: Freire
authors.forename: José Antonio
authors.initials: JA
authors.lastname: Vargas
authors.forename: Gerard
authors.initials: G
authors.lastname: Espinosa
mesh_terms: D000328:Adult; D017668:Age of Onset; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012307:Risk Factors; D012595:Scleroderma, Systemic / Q000150:complications* / Q000503:physiopathology*; D016896:Treatment Outcome