New insights into predictors of organ damage in childhood SLE

Childhood-onset systemic lupus erythematosus (cSLE) is a chronic, severe autoimmune disorder that carries a risk of early organ damage. Identifying specific prognostic factors in children is crucial to prevent such damage. At their 2024 conference, EULAR – The European Alliance of Societies for Rheumatology – held a session on pediatric rheumatology that presented new work on factors associated with lesion accumulation in cSLE, with an emphasis on corticosteroid regimens and maintaining low disease activity.

cSLE is a rare multisystem disorder with significant associated morbidity, but evidence-based guidelines are scarce and therefore management is often based on clinical experience. The EULAR/ACR-2019 criteria showed sensitivity in patients with cSLE, which could allow early identification of patients with single or major organ involvement, but identifying specific predictors in this vulnerable group is crucial to prevent long-term damage .

The new work, presented at the 2024 EULAR conference, aimed to investigate how clinical, demographic and treatment variables correlate with damage accumulation in cSLE. Maria Hanif and her colleagues hoped that stratifying patients according to average levels of disease activity over the course of the disease would help them identify independent predictors of damage – even in children with low disease activity.

To achieve this, data were collected on 430 children participating in the UK JSLE cohort study. Analyzes were performed in the entire cohort, as well as in two subgroups based on disease activity: low activity and moderate to high activity.

At a median follow-up period of 46 months, 23% of children experienced organ damage. In the entire cohort, multivariate analyzes showed that three factors were associated with lesion accumulation: methylprednisolone exposure, time-adjusted mean Physician Global Assessment (PGA) score, and Adjusted mean SLE Disease Activity Index (AMS) score. Looking only at the subgroup of moderate to high disease activity, 28.1% had damage – but the same three factors were identified as predictors. Within the low disease activity subgroup, 20.5% of children accumulated new lesions, and again methylprednisolone exposure and time-adjusted mean PGA score were associated with lesion accumulation, but not with AMS score.

This study highlights the role of corticosteroid exposure as an important and potentially modifiable risk factor for cSLE and suggests that there is a need to revise dosage limits for children—which typically exceed adult recommendations. Furthermore, a direct relationship was found between disease activity and impairment, with each 1-point increase in the SLE Disease Activity Index (SLEDAI) increasing the risk of impairment by 13–15% in moderately to highly active individuals. This was not seen in patients with AMS 4 or less, suggesting that low disease activity—maintained through targeted treatment strategies—could significantly reduce the risk of harm. These findings highlight the need for updated treatment protocols that limit corticosteroid use while effectively managing disease activity.