Longitudinal assessment of disease burden in juvenile systemic lupus erythematosus: A multicenter study of activity and damage scores


AYDIN D., Tunce E., KAVRUL KAYAALP G., Tüzen H. I., Alkan D., Oğuz G., ...More

Lupus, vol.34, no.13, pp.1398-1405, 2025 (SCI-Expanded, Scopus) identifier identifier

  • Publication Type: Article / Article
  • Volume: 34 Issue: 13
  • Publication Date: 2025
  • Doi Number: 10.1177/09612033251386091
  • Journal Name: Lupus
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus, Academic Search Premier, BIOSIS, MEDLINE
  • Page Numbers: pp.1398-1405
  • Keywords: corticosteroids, Juvenile systemic lupus erythematosus, LLDAS, organ damage, SLEDAI-2K, systemic lupus erythematosus
  • Çanakkale Onsekiz Mart University Affiliated: Yes

Abstract

Introduction: Juvenile Systemic Lupus Erythematosus (jSLE) is a rare pediatric rheumatic disease characterized by systemic inflammation that can lead to organ damage. Compared to adults, it often has a more severe course in children. Both disease activity and treatments may result in temporary or permanent damage. Objectives: To evaluate risk factors associated with damage occurrence in patients with jSLE. Methods: This multicenter, retrospective study included patients with jSLE followed for at least 12 months. Low-dose corticosteroid therapy was defined as prednisolone 0.01–0.03 mg/kg/day (max 7.5 mg/day). The annual cumulative steroid dose was calculated by dividing the total steroid intake by 365.25 times the number of follow-up years. Collected data included SLEDAI and SDI scores at initial and final visits, laboratory parameters, and flare characteristics. Results: A total of 158 patients (86.7% female) from 17 centers were included. Median age at diagnosis was 13.8 years, with a median follow-up of 35 months. Organ damage was present in 14 patients at diagnosis and in 23 at final visit. Damage types included proteinuria, cognitive dysfunction (each 3.2%), and others such as cataracts, erosive arthritis, avascular necrosis, optic atrophy, and vertebral collapse. Patients with damage had significantly higher SLEDAI scores at both time points, delayed transition to low-dose steroids, and a lower rate of achieving Lupus Low Disease Activity State (LLDAS) (p = .006). Conclusion: Persistent disease activity and delayed control are major contributors to organ damage in jSLE. Early and sustained disease suppression is critical to prevent long-term complications.