Siltuximab in Idiopathic Multicentric Castleman Disease: Real-World Experience
Notice bibliographique
Résumé
Background: Castleman disease (CD) is a very rare, non-malignant lymphoproliferative disorder that can be classified as unicentric or multicentric (MCD). MCD is associated with systemic symptoms, including organ dysfunction due to cytokine dysregulation, primarily interleukin-6 (IL-6). The anti-IL-6 monoclonal antibody siltuximab is recommended as a frontline treatment for idiopathic MCD (iMCD), but real-world data on its use in routine clinical practice are limited. This study aimed to assess disease response and survival outcomes in patients with iMCD treated with siltuximab therapy in real-world settings in Greece and Romania. Methods: This retrospective cohort study included adult patients with iMCD treated with siltuximab in clinical practice across Greece and Romania between January 2017 and December 2022. The primary endpoint was overall response rate and secondary endpoints included survival and safety outcomes. Response assessments were performed according to the Castleman Disease Collaborative Network guidelines. Patients were followed until death, loss to follow-up or study conclusion (October 2023). Results: Forty-eight patients with iMCD were included in the study. Mean age at baseline was 65 years, with significant age differences between patients from Greece (74 years) and Romania (54 years). The majority of patients were male (68.8%) and received one prior line of therapy (75%). Patients included in the study received a median of nine cycles of siltuximab. Response data were available for 38 patients. The overall response to siltuximab was 71.1%, with 55.3% of patients achieving a complete response, and 15.8% a partial response. The estimated overall survival rate at 3 years was 74% and the median survival was 123 months. The most common adverse events (> 5%) included elevated liver enzymes, anxiety, allergic reactions and nausea/diarrhea. Serious adverse events were experienced by 16.7% of the patients. Conclusions: Our results suggest that siltuximab-based therapy is effective in treating iMCD in real-world settings in Greece and Romania. To our knowledge, this study represents the largest real-world analysis of siltuximab in European patients with iMCD so far.
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 distillée sur la base complète
Imitation des enseignantsNi prévalence calibrée, ni vérité terrain. Validation humaine à venir. Apprise à partir de 10 348 étiquettes directes de Codex et de 10 348 étiquettes directes de Gemma. Le mode candidate est l'union des têtes enseignantes seuillées; le consensus est leur intersection. Ces sorties portent le statut machine_predicted_unvalidated et ne sont ni des étiquettes humaines ni des étiquettes directes de modèles de pointe.
Scores Codex et Gemma par catégorie
| Catégorie | Codex | Gemma |
|---|---|---|
| Métarecherche | 0,000 | 0,000 |
| Méta-épidémiologie (sens strict) | 0,000 | 0,000 |
| Méta-épidémiologie (sens large) | 0,000 | 0,000 |
| Bibliométrie | 0,000 | 0,001 |
| Études des sciences et des technologies | 0,000 | 0,000 |
| Communication savante | 0,000 | 0,000 |
| Science ouverte | 0,000 | 0,000 |
| Intégrité de la recherche | 0,000 | 0,000 |
| Charge utile insuffisante (le modèle a refusé de juger) | 0,000 | 0,000 |
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 tête enseignante, 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 ».