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AB0902 Pubescent Status-Related Differential Presentation in Pediatric-Onset Systemic Lupus Erythematosus: an Experience in the Single Center
  1. K.-S. Shin1,
  2. J.-S. Kim2,
  3. E.-J. Park3
  1. 1Pediatrics
  2. 2Internal Medicine, Jeju National University School of Medicine
  3. 3Internal Medicine, Jeju National University Hospital, Jeju, Korea, Republic Of

Abstract

Background Pediatric-onset SLE (pSLE) represents 10-20% of all SLE patients, and several studies suggest that SLE tends to be more severe in pSLE than in adult-onset SLE. However, the impact of age or puberty at disease onset in children remains unclear and has rarely been studied.

Objectives To evaluate the association between pubescent status and the initial presentation of pSLE.

Methods Patients with a diagnosis of SLE before the age of 16 years and followed up at Jeju National University Hospital from 2003 to 2013 were eligible for inclusion in this study. Patients were divided into two groups, based on pubertal stage (> Tanner stage II) and/or menarche at diagnosis, defined as pre-pubescent and pubescent. Medical records regarding demographic profile, clinical and laboratory manifestations at diagnosis, SLE Disease Activity Index (SLEDAI) at diagnosis were reviewed.

Results Forty-two patients with pSLE were included in this study: 36 girls (85.7%) and 6 boys (14.3%). The median age at diagnosis was 12.4 years (range 7.8 to 15.6 years). The cohort of pre-pubescent patients consisted of 9 girls and 5 boys (83.3% in boys), and the most of pubescent patients were girls (27, 96.4% in pubescent). Pre-pubescent patients showed significantly more hematologic and constitutional manifestations at diagnosis compared with pubescent patients, but musculoskeletal, cutaneous, and neurologic involvement occurred more frequently in pubescent patients. Renal involvement, complement level, and autoantibodies at diagnosis were not significantly different between the two cohorts. Pubescent patients showed a significantly higher disease activity score than pre-pubescent patients.

Conclusions Although the number of patient was so small, these results suggest that the disease activity at diagnosis in pSLE significantly increased after pubescent and the distinct differences in the initial presentation between pre-pubescent and pubescent patients relate to musculoskeletal, cutaneous, and neurologic manifestations.

References

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  2. Descloux E, Durieu I, Cochat P, Vital-Durand D, Ninet J. Influence of age at disease onset in the outcome of paediatric systemic lupus erythematosus. Rheumatol 2009;48:779-84.

  3. Hui-Yuen JS, Imundo LF, Avitabile C, Kahn PJ, Eichenfield AH, Levy DM. Early versus later onset childhood-onset systemic lupus erythematosus: Clinical features, treatment and outcome. Lupus 2011;20:952-9.

  4. Malattia C, Martini A. Paediatric-onset systemic lupus erythematosus. Best Prac Res Clin Rheumatol 2013;27:351-62.

Disclosure of Interest None declared

DOI 10.1136/annrheumdis-2014-eular.4172

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