CHILDHOOD-ONSET SYSTEMIC LUPUS ERYTHEMATOSUS – SINGLE CENTER’S 10-YEAR EXPERIENCE
Notice bibliographique
Résumé
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.
Récupéré en direct depuis OpenAlex et désinversé. Les résumés ne sont pas conservés dans cette base de données : les index inversés représentent 8,6 Go des 9,3 Go de texte de la base, et le serveur dispose de 13 Go libres.
Comment cette classification a été obtenuedéplier
Prédiction machine sur la base complète
Imitation des enseignantsNi prévalence calibrée, ni vérité terrain. Validation humaine à venir. Le volet Gemma est une étiquette directe du modèle pour chaque travail de la base, lue sur la notice réduite au titre. Le volet Codex est un classifieur appris des 10 348 étiquettes directes de Codex et calibré sur les taux pondérés de l'échantillon; les champs sans appui suffisant ne portent aucun appel Codex. Le mode candidate est l'union des deux volets; le consensus est leur intersection. Ces sorties portent le statut machine_predicted_unvalidated et ne sont pas des étiquettes humaines.
Scores du classifieur distillé par catégorie (deux têtes)
| Catégorie | Codex | Gemma |
|---|---|---|
| Métarecherche | 0,000 | 0,001 |
| Méta-épidémiologie (sens strict) | 0,000 | 0,000 |
| Méta-épidémiologie (sens large) | 0,001 | 0,000 |
| Bibliométrie | 0,001 | 0,001 |
| Études des sciences et des technologies | 0,001 | 0,001 |
| Communication savante | 0,001 | 0,001 |
| Science ouverte | 0,000 | 0,001 |
| Intégrité de la recherche | 0,001 | 0,001 |
| Charge utile insuffisante (le modèle a refusé de juger) | 0,002 | 0,001 |
Scores machine (provisoires)
Les deux têtes enseignantes du modèle étudiant, lues sur ce travail. Un score ordonne la base pour la relecture; il n'affirme jamais une catégorie, et le statut de validation accompagne chaque rangée tel quel.
Scores de référence d'un modèle non mature (critères de maturité non atteints, 7 itérations). Un score ordonne; il n'affirme jamais une catégorie.
score_only:v0-immature-baseline · tel quel depuis la passe de notation : score_only signifie que le nombre peut ordonner les travaux, et qu'aucune étiquette de catégorie n'en découleClassification
machine, non validéePrédiction automatique; un appel candidat d’une seule source (Gemma direct ou Codex distillé), pas un consensus.
Le détail, modèle par modèle et score par score, se trouve en fin de page sous « Comment cette classification a été obtenue ».