AB0516 Thrombotic Thrombocytopenic Purpura in Patients from the SLE Registry of the Spanish Society of Rheumatology (Relesser)
| Autor: | Loreto Horcada, J. deToro, Enrique Raya, J.M. Pego, Mercedes Freire, Paloma Vela, Eva Tomero, A. Lois, Carlos Montilla, Roman Blanco, V. delCampo, C. Erausquin, A. Fernández, Isabel Hernández, I. Rúa, M. Belmonte, María Galindo, F. Lόpez, E. Uriarte, V. Calvo, J. Calvo |
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| Rok vydání: | 2015 |
| Předmět: |
medicine.medical_specialty
Pediatrics Systemic lupus erythematosus Cyclophosphamide Anemia business.industry Immunology Thrombotic thrombocytopenic purpura medicine.disease General Biochemistry Genetics and Molecular Biology Rheumatology Serology Internal medicine Cohort medicine Immunology and Allergy In patient business medicine.drug |
| Zdroj: | Annals of the Rheumatic Diseases. 74:1072.2-1073 |
| ISSN: | 1468-2060 0003-4967 0961-2033 |
| DOI: | 10.1136/annrheumdis-2015-eular.2779 |
| Popis: | Background In our area, SLE patients present haematologic manifestations in approximately 70% of cases1. Some of these manifestations are rare so there are no large series whose analysis can provide relevant information. Objectives To study the characteristics of patients with thrombotic thrombocytopenic purpura (TTP) in a large sample of SLE patients. Methods SLE patients from RELESSER database were studied. We analysed the clinical and serological SLE manifestations present at the 9 domains of the British Isles Lupus Assessment Group (BILAG) activity index, all of them before, during and after TTP diagnosis until the last patient's assessment. We also studied activity (SELENA-SLEDAI) and damage (SLICC/ACR DI) indices in each one of those moments. We evaluated the treatment received, TTP recurrences and the number of deaths by this entity. Results 3,656 patients from 45 Rheumatology Units across Spain were studied. We found 19 cases of PTT (6). Afterwards the patients maintained SLEDAI scores of 3.8±5.3 at G2 and 2.4±0.7 at G3. The number of therapy lines and the number of treatments administered was 2.8±2 and 5.4±2.9, respectively in G2, and 2.4±1.7 and 3.8±2.5 in G3, contrasting with G1 where the patients needed only 1 line ±0 of treatment and 2±1 drugs. The treatment most frecuently received by patients in G1 were glucocorticoids (GC)(100%), in G2 GC (100%), plasma exchange (PE) (80%) and cyclophosphamide (CyC)(60%); in G3 GC (81.8%), PE (72.7%) and CyC (41.8%). There were only recurrences and deaths in G3, 2 patients in each case. More detailed results of the analysis in each of the 3 groups are shown in [table 1][1]. ![Figure][2] Conclusions TTP is a rare event ( |
| Databáze: | OpenAIRE |
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