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Childhood versus adult-onset systemic lupus erythematosus: long-term outcome and predictors of mortality

Clinical Rheumatology volume 36, pages 343–350 (2017)Cite this article

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.

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Affiliations

  1. Department of Rheumatology, Alzahra Hospital, Sofe Street, Isfahan University of Medical Sciences, Isfahan, Iran

    Alimohammad Fatemi

  2. Department of Internal Medicine, Alzahra Hospital, Sofe Street, Isfahan University of Medical Sciences, Isfahan, Iran

    Mohammad Matinfar

  3. Department of Epidemiology and Biostatistics, School of Public Health, Indiana University, Bloomington, IN, USA

    Abbas Smiley

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  1. Alimohammad Fatemi
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Correspondence to Alimohammad Fatemi.

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Fatemi, A., Matinfar, M. & Smiley, A. Childhood versus adult-onset systemic lupus erythematosus: long-term outcome and predictors of mortality. Clin Rheumatol 36, 343–350 (2017). https://doi.org/10.1007/s10067-016-3509-1

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  • DOI: https://doi.org/10.1007/s10067-016-3509-1

Keywords

  • Adult
  • Children
  • Outcome
  • Survival
  • Systematic lupus erythematosus