Characterizing juvenile-onset systemic lupus erythematosus: clinical outcomes, disease progression, and determinants in pediatric populations.

Abstract

Introduction: This study aims to delineate the sociodemographic, clinical, and laboratory characteristics of juvenile-onset systemic lupus erythematosus (jSLE) patients.

Materials and methods: Retrospective data from jSLE patients treated at Ümraniye Training and Research Hospital's pediatric rheumatology unit between January 2017 and February 2024 were collected. Inclusion criteria comprised meeting at least four of the American College of Rheumatology (ACR) criteria for systemic lupus erythematosus (SLE) classification and being under 18 years old at disease onset.

Results: The study encompassed 69 jSLE patients, with a female-to-male ratio of approximately 3.9:1 and a median diagnosis age of 14.5 (min:2, max:17.5) years. Musculoskeletal symptoms, nephropathy, malar rash, and hematologic abnormalities were predominant clinical features. Thirty-seven patients exhibited renal involvement, 36 presented hematological complications, and 23 had both. Overall, 76.8% of patients demonstrated major organ system involvement. A statistically significant association was observed between renal involvement and initial Anti-double-stranded deoxyribonucleic acid antibody presence (p=0.036) and SLE Disease Activity Index-2000 (SLEDAI-2K) scores (p=0 and p=0.003 at diagnosis and latest visit). Significant associations were observed between follow-up duration, SLEDAI-2K at first visit scores, treatment modalities (pulse methylprednisolone, mycophenolate mofetil and rituximab), and remission subtypes in patients with jSLE. Shorter follow-up periods and lower initial SLEDAI-2K scores were linked to better remission outcomes.

Conclusions: This study found that jSLE mainly affects female patients, with musculoskeletal, renal, and hematologic involvement being the most common manifestations. Renal involvement is associated with initial anti-dsDNA positivity and SLEDAI-2K scores. The study also found that better remission outcomes are linked to lower initial disease activity and longer follow-up periods.