ECULIZUMAB IN THE MANAGEMENT OF CATASTROPHIC ANTIPHOSPHOLIPID SYNDROME WITH MULTIORGAN INVOLVEMENT: A CASE REPORT
Notice bibliographique
Résumé
PV306 / #753 Case Report Poster Topic: AS03 - Antiphospholipid Syndrome Late-Breaking Abstract Introduction Catastrophic antiphospholipid syndrome (CAPS) is a severe and rapidly progressive form of antiphospholipid syndrome that results in multiorgan failure due to thrombosis in the microvasculature. It is rare, occurring in only 1% of patients with antiphospholipid syndrome, but it carries a high mortality rate and can be the first manifestation of the disease. Eculizumab, a C5-blocking monoclonal antibody, is effective in the treatment of thrombotic microangiopathy by controlling complement system hyperactivation. Due to the rarity of CAPS, there is limited literature regarding the appropriate management of these patients, and there is little clinical experience with Eculizumab treatment. This case report aims to provide clinical insight to improve the management of patients with catastrophic antiphospholipid syndrome and multiorgan involvement. Case Presentation With Investigation We present the case of a 62-year-old female patient with a debut diagnosis of CAPS secondary to systemic lupus erythematosus. She exhibited renal involvement with significant deterioration of kidney function, which required hemodialysis. Renal biopsy revealed thrombotic microangiopathy affecting small and medium-sized vessels and glomeruli. Additionally, she exhibited cardiopulmonar involvement, including nonischemic dilated cardiomyopathy and pulmonary hypertension. Laboratory findings showed hemolytic anemia, thrombocytopenia, renal failure, complement consumption, and positivity for ANA, anticardiolipin IgM, and beta-2 glycoprotein antibodies. In the first weeks following the initial diagnosis, the patient received treatment with hydroxychloroquine (200 mg every 12 hours), methylprednisolone boluses (500 mg for 3 days), followed by prednisone at a tapering dose, immunoglobulin therapy (weight-adjusted for 5 days), mycophenolate (500 mg every 12 hours), and 3 cycles of Rituximab 1 g. Anticoagulation was managed with bemiparin. Additionally, treatment with Eculizumab was initiated with 4 weekly doses, followed by Eculizumab every 2 weeks for 3 months. It was interrupted due to the patient’s admission for bacteremia, with a good clinical outcome. Afterward, it was not reintroduced as the patient remained stable with her disease and showed very good clinical progress, as well as normalization of laboratory values, with no significant incidents during 18 months. Literature Review [1.] Almeida C. Autoimmun Rev 2015;14:1087-96. [2.] Guerra N. Acta Colombiana de Cuidado Intensivo 2019;19:154-9. Discussion Given the severity and high mortality of CAPS, early diagnosis and appropriate treatment are critical to prevent irreversible structural damage to target organs. Early treatment with anticoagulation and intravenous corticosteroids, followed by plasmapheresis, rituximab, and eculizumab in the early stages, is crucial to achieve disease remission and prevent irreversible structural damage to target organs.
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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 | 0,001 | 0,000 |
| Méta-épidémiologie (sens strict) | 0,000 | 0,000 |
| Méta-épidémiologie (sens large) | 0,000 | 0,000 |
| Bibliométrie | 0,000 | 0,000 |
| 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.
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