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2014 ; 93
(2
): 73-81
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English Wikipedia
Early- versus late-onset systemic sclerosis: differences in clinical presentation
and outcome in 1037 patients
#MMPMID24646463
Alba MA
; Velasco C
; Simeón CP
; Fonollosa V
; Trapiella L
; Egurbide MV
; Sáez L
; Castillo MJ
; Callejas JL
; Camps MT
; Tolosa C
; Ríos JJ
; Freire M
; Vargas JA
; Espinosa G
Medicine (Baltimore)
2014[Mar]; 93
(2
): 73-81
PMID24646463
show ga
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.