Research Information System
32nd European Paediatric Rheumatology Congress, Helsinki, Finland, 17 - 20 September 2025, pp.187-188, (Summary Text)
Introduction: Juvenile Systemic Lupus Erythematosus (jSLE)
is a rare pediatric rheumatic disease marked by systemic inflammation that can
cause organ damage. Compared to adults, it often follows a more severe course
in children. Corticosteroids are widely used for their rapid antiinflammatory
effects; however, their adverse events have made steroidsparing strategies
increasingly important in disease management. Objectives: This study aimed to
evaluate the impact of corticosteroid use on disease activity and damage index
in patients diagnosed with jSLE. Methods: This was a multicenter, retrospective
study including patients who were followed for at least 12 months due to jSLE.
Lowdose corticosteroid therapy was defined as 0.01–0.03 mg/kg/day (maximum 7.5
mg/day) of prednisolone. The annual cumulative steroid dose (mg/day) was
calculated by dividing the total amount of steroid received on treatment days
by the product of 365.25 and the number of years. Data collected included SLE
Disease Activity Index (SLEDAI) and Systemic Lupus International Collaborating
Clinics/American College of Rheumatology Damage Index (SDI) at the initial and
final visits, laboratory findings, and flare characteristics Results: A total
of 138 patients from 11 centers were enrolled. Of these, 121 (87.7%) were
female and 17 (12.3%) were male. The median age at diagnosis was 14 years
(range: 2–16), and the median follow-up period was 36 months (range: 12–170).
At diagnosis, 21 patients had organ damage, while 19 had organ damage at their
last visit. Of the 10 patients who had damage at diagnosis, damage persisted in
the final visit. Observed damage included cataracts (n=5, 3.6%), nephroticrange
proteinuria (n=5, 3.6%), erosive arthritis (n=4, 2.9%), cognitive dysfunction
(n=3, 2.3%), avascular necrosis (n=2, 1.4%), and one patient each (0.7%) with
decreased GFR (<50%), retinal changes or optic atrophy, venous thrombosis,
pancreatic insufficiency, stroke, transverse myelitis, end-stage renal disease,
pulmonary hypertension, pleural fibrosis, reduced lung capacity, previous
myocardial infarction, valvular involvement, muscle atrophy, vertebral height
loss, and scarring alopecia. Although follow-up durations were similar between
groups, patients with damage had higher SLEDAI scores at both initial and final
visits and took longer to achieve low-dose steroid therapy. Additionally, the
proportion of patients who achieved Lupus Low Disease Activity State (LLDAS)
was significantly lower in the damage group (p=0.02). Conclusion: Organ damage
in jSLE is associated with increased morbidity and mortality. Identifying and
mitigating factors that contribute to long-term damage is essential. While
glucocorticoids are effective and accessible agents for disease control, they
should be used at the lowest effective dose and for the shortest duration
possible to minimize adverse outcomes.
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