Evaluation of disease activity and damage index in patients diagnosed with juvenile systemic lupus erythematosus

<p class="MsoNormal">Introduction: Juvenile Systemic Lupus Erythematosus (jSLE)is a rare pediatric rheumatic disease marked by systemic inflammation that cancause organ damage. Compared to adults, it often follows a more severe coursein children. Corticosteroids are widely used for their rapid antiinflammatoryeffects; however, their adverse events have made steroidsparing strategiesincreasingly important in disease management. Objectives: This study aimed toevaluate the impact of corticosteroid use on disease activity and damage indexin patients diagnosed with jSLE. Methods: This was a multicenter, retrospectivestudy 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 7.5mg/day) of prednisolone. The annual cumulative steroid dose (mg/day) wascalculated by dividing the total amount of steroid received on treatment daysby the product of 365.25 and the number of years. Data collected included SLEDisease Activity Index (SLEDAI) and Systemic Lupus International CollaboratingClinics/American College of Rheumatology Damage Index (SDI) at the initial andfinal visits, laboratory findings, and flare characteristics Results: A totalof 138 patients from 11 centers were enrolled. Of these, 121 (87.7%) werefemale 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 theirlast visit. Of the 10 patients who had damage at diagnosis, damage persisted inthe final visit. Observed damage included cataracts (n=5, 3.6%), nephroticrangeproteinuria (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%) withdecreased GFR (&lt;50%), retinal changes or optic atrophy, venous thrombosis,pancreatic insufficiency, stroke, transverse myelitis, end-stage renal disease,pulmonary hypertension, pleural fibrosis, reduced lung capacity, previousmyocardial infarction, valvular involvement, muscle atrophy, vertebral heightloss, and scarring alopecia. Although follow-up durations were similar betweengroups, patients with damage had higher SLEDAI scores at both initial and finalvisits and took longer to achieve low-dose steroid therapy. Additionally, theproportion of patients who achieved Lupus Low Disease Activity State (LLDAS)was significantly lower in the damage group (p=0.02). Conclusion: Organ damagein jSLE is associated with increased morbidity and mortality. Identifying andmitigating factors that contribute to long-term damage is essential. Whileglucocorticoids are effective and accessible agents for disease control, theyshould be used at the lowest effective dose and for the shortest durationpossible to minimize adverse outcomes. <o:p></o:p> <p class="MsoNormal">References 1. Hanif, M., Sarker, C., Al-Abadi, E., Armon,K., Bailey, K., Bohm, M., Brennan, M., Ciurtin, C., Gardner-Medwin, J., Hawley,D. P., Kinder, A., Leahy, A., Malik, G., McLaren, Z., Moraitis, E., Mosley, E.,Ramanan, A. V., Rangaraj, S., Ratcliffe, A., Riley, P., … Smith, E. M. D.(2025). Contributors to organ damage in childhood lupus: corticosteroid use anddisease activity. Rheumatology (Oxford, England), 64(5), 3028–3038. 2.Davidson, J. E., Fu, Q., Rao, S., Magder, L. S., &amp; Petri, M. (2018). Quantifyingthe burden of steroid-related damage in SLE in the Hopkins Lupus Cohort. Lupusscience &amp; medicine, 5(1), e000237.<o:p></o:p>