Novel insights into predictors of organ damage in childhood-onset SLE

Childhood-onset systemic lupus erythematosus (cSLE) is a chronic, severe autoimmune disease that carries the risk of early organ damage. Identifying specific predictors in children is critical to preventing such damage. At the 2024 Congress, EULAR – the European Association of Societies for Rheumatology – held a session on pediatric rheumatology, presenting modern work on factors associated with the progression of damage in cSLE, with particular emphasis on corticosteroid treatment regimens and maintaining low disease activity.

cSLE is a uncommon multisystem disease with significant morbidity, but evidence-based guidelines are meager and treatment is often based on clinical knowledge. The EULAR/ACR-2019 criteria have demonstrated sensitivity in patients with cSLE, which could enable earlier diagnosis of patients with single or major organ involvement, but identifying specific predictive factors in this vulnerable group is crucial to prevent long-term damage.

The aim of the modern work, presented at the 2024 EULAR Congress, was to investigate how clinical, demographic and treatment variables correlate with the progression of damage in cSLE. Maria Hanif and colleagues hoped that stratifying patients by average level of disease activity over the course of their disease would lend a hand them identify independent predictors of damage – even in children with low disease activity.

To achieve this, data was collected from 430 children taking part 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.

Over an average follow-up period of 46 months, 23% of children developed organ damage. Within the entire cohort, multivariate analyzes showed that three factors were associated with increasing damage: methylprednisolone exposure, time-adjusted mean Physician Global Assessment (PGA) score, and adjusted mean SLE Disease Activity Score (AMS). Looking only at the subgroup of people with moderate to high disease activity, 28.1% experienced damage, but the same three factors were found to be predictive factors. In the low disease activity subgroup, 20.5% of children developed modern lesions and, again, methylprednisolone exposure and time-adjusted mean PGA score were associated with increasing lesions but not with AMS score.

This study highlights the role of corticosteroid exposure as an crucial and potentially modifiable risk factor in cSLE and suggests that there is a need to review dose limits used in children, which typically exceed recommendations for adults. Additionally, a direct relationship has been found between disease activity and damage, with each 1-unit enhance in the SLE Disease Activity Index (SLEDAI) increasing the risk of damage by 13–15% in people with moderate to high activity. This was not observed in patients with an AMS of 4 or less, suggesting that low disease activity – maintained through goal-directed treatment strategies – can significantly reduce the risk of damage. These findings highlight the need for updated treatment protocols that limit the apply of corticosteroids while effectively controlling disease activity.