CHILDHOOD-ONSET SYSTEMIC LUPUS ERYTHEMATOSUS – SINGLE CENTER’S 10-YEAR EXPERIENCE
Bibliographic record
Abstract
PV165 / #571 Poster Topic: AS18 - Pediatric SLE Background/Purpose Childhood-onset systemic lupus erythematosus (cSLE) is a rare, chronic autoimmune multisystem inflammatory disease that manifests before the age of 16 years. The course of the disease in children is more severe than in adults. The clinical presentation of cSLE is multifaceted, most commonly including constitutional, hematological, cutaneous, renal, and neuropsychiatric symptoms. The aim of the study was to characterize cSLE cohort in single center in Poland in years: 2014-2024. Methods A total of 35 Caucasian children (27 girls, 8 boys) diagnosed with cSLE according to SLICC 2012 criteria in Department of Pediatric Cardiology and Rheumatology Medical University of Lodz, Poland, were included in the retrospective analysis. Clinical features, laboratory and imaging test, as well as, kidney biopsy if necessary and treatment strategies were analyzed. Results The female-to-male ratio was 3.37:1. The median age at diagnosis was 14 years (7.5 to 18). The most common clinical symptoms were photosensitivity/malar rash/ (15/35 – 42.8%), oral ulcers (11/35 – 31.4%) and arthritis (9/35 – 25.7%) (Table 1). The severe course of cSLE was observed in 18/35 (51.4%) children. Central nervous system was affected in 14/35 (40%) children – 5 (14.3%) complained of headaches, 2 (5.7%) - depression, 2 (5.7%) – epilepsy. Proteinuria (>500 mg/24h) associated with histopathological lupus nephritis (3- stage IV, 1- stage - II) was observed in 4/35 (11.4%) patients. In 9/35 (25.7%) children leukopenia was observed, 5 /35 (14.3%) children have anemia and the same number thrombocytopenia. Antinuclear antibodies were positive (titer >1:80) in all children with cSLE, and above 1:320 in 27/35 (77.1%) patients. Fourteen (40%) children had dsDNA antibodies; 2/35 (5.7%) patients with lupus nephritis were anti-dsDNA positive. Anti-phospholipid antibodies were detected in 8/35 (22.8%) children, including 3 patients with neuropsychiatric manifestations, and 1 child with neuropsychiatric symptoms had also anti-Sm antibodies (Table 2). Low total activity of compliment (CH50) was found in 14/35 (40%) patients with cSLE. Six patients had Raynaud symptom and only 3/35 (8.6%) had microangiopathy confirmed by capillaroscopy. All the patients with cSLE were treated with hydroxychloroquine and supplemented with vitamin D. Twenty-three children (65.7%) required systemic corticosteroids, included 13/35 (37.1%) patients who received methylprednisolone intra venous pulses followed by oral prednisone. Seven children (20%) were treated with IVIG. Mycophenolane mophetyl was used in 8/35 (22.8%) cases, methotrexate - in 4/35 (11.4%). The observed complications were related to the prolonged use of glucocorticoids - cushingoid symptoms, such as obesity and fat tissue redistribution and stretch marks. Table 1. Clinical symptoms observed in study group of children with cSLE. Table 2. Immunological markers in study group of children with cSLE. Conclusions Our study confirms that the course of cSLE is severe. Neuropsychiatric symptoms are common in children population. Lupus nephritis requires the administration of steroids, which can be associated with adverse effects.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.001 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.000 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.000 | 0.001 |
| Research integrity | 0.001 | 0.001 |
| Insufficient payload (model declined to judge) | 0.002 | 0.001 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".