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The bm12 Inducible Model of Systemic Lupus Erythematosus (SLE) in C57BL/6 Mice
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The bm12 Inducible Model of Systemic Lupus Erythematosus (SLE) in C57BL/6 Mice

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Disease activity, severity, and damage in the UK Juvenile-Onset Systemic Lupus Erythematosus Cohort

Louise Watson1, Valentina Leone, Clarissa Pilkington

  • 1University of Liverpool and Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, UK.

Arthritis and Rheumatism
|February 2, 2012
PubMed

Insights

Juvenile-onset systemic lupus erythematosus (JSLE) in the UK is characterized by severe organ involvement and high disease activity. This national cohort study highlights significant clinical manifestations and disease progression in affected children.

Area of Science:

  • Rheumatology
  • Pediatric Autoimmunity
  • Clinical Epidemiology

Background:

  • Juvenile-onset systemic lupus erythematosus (JSLE) requires better understanding of its clinical spectrum and disease course.
  • The UK Juvenile-Onset Systemic Lupus Erythematosus (JSLE) Cohort Study was established to address this knowledge gap.

Purpose of the Study:

  • To describe the clinical manifestations and disease course of patients with JSLE.
  • To analyze data from a large, national inception cohort of children with JSLE.

Main Methods:

  • A multicenter collaborative network collected detailed clinical data from 232 patients over 4.5 years.
  • Patients were identified using the American College of Rheumatology (ACR) SLE classification criteria.
  • The cohort included 198 children meeting ≥4 ACR criteria for SLE.

Main Results:

  • The female:male sex ratio was 5.6:1, with a median age at diagnosis of 12.6 years.
  • Non-Caucasian UK patients showed a greater risk of JSLE.
  • High frequencies of musculoskeletal (82%), renal (80%), hematologic (91%), immunologic (54%), and neurologic (26%) involvement were observed, with 93% on steroids and 28% experiencing disease damage.

Conclusions:

  • JSLE is characterized by severe organ involvement and significant disease activity in children.
  • Accumulation of disease-associated damage is a notable feature in pediatric patients with JSLE.
Abstract

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