title: Childhood versus adult-onset systemic lupus erythematosus: long-term outcome and predictors of mortality.
abstract: The aim of this study was to compare survival of childhood-onset systemic lupus erythematosus (c-SLE) and adult-onset SLE (a-SLE) according to initial manifestations. This was a retrospective cohort study. All patients were categorized into c-SLE (≤18 years) and a-SLE (>18 years). The clinical and serological data at the time of diagnosis were recorded and compared. Kaplan-Meier curves were used to compare survival rates between the two groups. Predictors of mortality were obtained by a backward Cox regression. One hundred eighty patients with c-SLE and 394 patients with a-SLE were enrolled. The female/male ratio was higher in c-SLE (P = 0.0001). Lupus nephritis (P = 0.002) and valvular heart disease (P = 0.025) were more common in c-SLE and a-SLE, respectively. In a 23-year follow-up, 20 patients (11.1%) with c-SLE and 35 patients (8.9%) with a-SLE died. Mortality was not significantly different between them (P = 0.4). The main causes of death were nephritis (50% in c-SLE vs. 29% in a-SLE), infections (40% in c-SLE vs. 29% in a-SLE), and circulatory disease (10% in c-SLE vs. 37% in a-SLE). The difference was not significant (P = 0.08). Cumulative survival rates after 5, 10, 15, and 20 years were 91, 87, 85, and 78% in c-SLE and 93, 90, 90, and 83% in a-SLE, respectively. By multivariate analysis, seizure, proteinuria, and nephritis in c-SLE and seizure, hematuria, and pericarditis in a-SLE had negative prognostic effect on survival. Both c-SLE and a-SLE patients with seizure or renal involvement should be monitored more carefully to prevent ominous outcomes.
authors.affiliation: Department of Rheumatology, Alzahra Hospital, Sofe Street, Isfahan University of Medical Sciences, Isfahan, Iran. a_fatemi@med.mui.ac.ir.
authors.forename: Alimohammad
authors.initials: A
authors.lastname: Fatemi
authors.affiliation: Department of Internal Medicine, Alzahra Hospital, Sofe Street, Isfahan University of Medical Sciences, Isfahan, Iran.
authors.forename: Mohammad
authors.initials: M
authors.lastname: Matinfar
authors.affiliation: Department of Epidemiology and Biostatistics, School of Public Health, Indiana University, Bloomington, IN, USA.
authors.forename: Abbas
authors.initials: A
authors.lastname: Smiley
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D002648:Child; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008180:Lupus Erythematosus, Systemic / Q000453:epidemiology / Q000401:mortality* / Q000628:therapy*; D008297:Male; D011379:Prognosis; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D016896:Treatment Outcome
keywords: Adult; Children; Outcome; Survival; Systematic lupus erythematosus