Immune-mediated thrombotic thrombocytopenic purpura with systemic lupus erythematosus: clinical features and outcome
Objective The association of immune-mediated thrombotic thrombocytopenic purpura (iTTP) and SLE was previously described, but patients with iTTP with coexistent SLE remain poorly characterised.Methods We compared the clinical presentation and the outcome of patients with iTTP with coexistent SLE (SL...
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BMJ Publishing Group
2025-07-01
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| Series: | Lupus Science and Medicine |
| Online Access: | https://lupus.bmj.com/content/12/2/e001691.full |
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| author | David Ribes Nihal Martis Jehane Fadlallah Ygal Benhamou Veronique Le Guern Virginie Rieu Gabriel Choukroun Raïda Bouzid Agnès Veyradier Paul Coppo Bérangère S Joly Júlia Weisinger François Provôt Pascale Poullin Yahsou Delmas Sylvain Chantepie Manon Marie Ranta Dana |
| author_facet | David Ribes Nihal Martis Jehane Fadlallah Ygal Benhamou Veronique Le Guern Virginie Rieu Gabriel Choukroun Raïda Bouzid Agnès Veyradier Paul Coppo Bérangère S Joly Júlia Weisinger François Provôt Pascale Poullin Yahsou Delmas Sylvain Chantepie Manon Marie Ranta Dana |
| author_sort | David Ribes |
| collection | DOAJ |
| description | Objective The association of immune-mediated thrombotic thrombocytopenic purpura (iTTP) and SLE was previously described, but patients with iTTP with coexistent SLE remain poorly characterised.Methods We compared the clinical presentation and the outcome of patients with iTTP with coexistent SLE (SLE-iTTP) to an age-sex matched cohort of patients with idiopathic iTTP without SLE.Results During the study period, 1409 patients with iTTP were recruited in our registry. Of these, 79 (6%) had a prior or concurrent diagnosis of SLE at the time of iTTP diagnosis, and 437 (31%) had detectable ANAs without other clinical features of SLE. When compared with idiopathic iTTP, patients with SLE-iTTP had more severe renal involvement, and cardiac involvement was more prevalent, whereas central nervous system involvement was less common. Patients with SLE-iTTP received more immunosuppressive agents. There was no difference in response categories during the acute phase. During follow-up, SLE-iTTP had superior ADAMTS13 relapse-free survival than idiopathic iTTP. Among patients with ANAs without other clinical features of SLE, 33 (8%) were diagnosed with SLE 27 months (IQR: 7–65 months) following iTTP diagnosis; 32 additional patients (7%) developed another systemic autoimmune disease. No patient from the idiopathic iTTP group developed clinical SLE during follow-up.Conclusion Patients with iTTP are prone to develop autoimmune features, and patients with SLE-iTTP have distinct clinical features and outcome. Relapse-free survival seems better in patients with SLE-iTTP, underscoring the need for tailored management strategies in this population, including a specific follow-up to assess early features suggestive of SLE.Trial registration number NCT00426686. |
| format | Article |
| id | doaj-art-214fc2559b7c48d9a8e00a352bf33b12 |
| institution | Kabale University |
| issn | 2053-8790 |
| language | English |
| publishDate | 2025-07-01 |
| publisher | BMJ Publishing Group |
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| series | Lupus Science and Medicine |
| spelling | doaj-art-214fc2559b7c48d9a8e00a352bf33b122025-08-20T03:56:05ZengBMJ Publishing GroupLupus Science and Medicine2053-87902025-07-0112210.1136/lupus-2025-001691Immune-mediated thrombotic thrombocytopenic purpura with systemic lupus erythematosus: clinical features and outcomeDavid Ribes0Nihal Martis1Jehane Fadlallah2Ygal Benhamou3Veronique Le Guern4Virginie Rieu5Gabriel Choukroun6Raïda Bouzid7Agnès Veyradier8Paul Coppo9Bérangère S Joly10Júlia Weisinger11François Provôt12Pascale Poullin13Yahsou Delmas14Sylvain Chantepie15Manon Marie16Ranta Dana17INSERM U1297, Department of Nephrology and Organ Transplantation, Referral Centre for Rare Kidney Diseases, University Hospital of Toulouse, Toulouse, FranceService de Médecine Interne et d’Immunologie Clinique, Hopital de Nice, Nice, FranceService d’Immunologie Clinique, Hôpital Saint-Louis, APHP, Paris, FranceService de Médecine Interne, CHU Charles Nicolle, Rouen, FranceUnité d’Hémaphérèse, Service de Médecine Interne, Hôpital Cochin, Paris, FranceService de Médecine Interne, CHU de Clermont-Ferrand, Clermont-Ferrand, FranceService de Néphrologie Dialyse et Transplantation, CHU d’Amiens et UPJV, Amiens, FranceCentre de Référence des Microangiopathies Thrombotiques et Service d’hématologie, Hôpital Saint Antoine, Assistance Publique-Hôpitaux de Paris, Sorbonne Université, Paris, FranceCentre de Référence des Microangiopathies Thrombotiques et Service d’hématologie, Hôpital Saint Antoine, Assistance Publique-Hôpitaux de Paris, Sorbonne Université, Paris, FranceCentre de Référence des Microangiopathies Thrombotiques et Service d’hématologie, Hôpital Saint Antoine, Assistance Publique-Hôpitaux de Paris, Sorbonne Université, Paris, FranceCentre de Référence des Microangiopathies Thrombotiques et Service d’hématologie, Hôpital Saint Antoine, Assistance Publique-Hôpitaux de Paris, Sorbonne Université, Paris, FranceCentre de Référence des Microangiopathies Thrombotiques et Service d’hématologie, Hôpital Saint Antoine, Assistance Publique-Hôpitaux de Paris, Sorbonne Université, Paris, FranceService de Néphrologie, Hôpital Albert Calmette, Lille, FranceService d’Hémaphérèse, Hôpital de La Conception, CHU de Marseille, Marseille, FranceService de Néphrologie, CHU Bordeaux, Bordeaux, FranceInstitut d’Hématologie de Basse Normandie, Centre Hospitalier Universitaire, Caen, FranceService de Médecine Interne, Hôpital Edouard Herriot, Hospices Civils de Lyon, Lyon, FranceService d’Hématologie, Centre Hospitalier Universitaire de Nancy, Nancy, FranceObjective The association of immune-mediated thrombotic thrombocytopenic purpura (iTTP) and SLE was previously described, but patients with iTTP with coexistent SLE remain poorly characterised.Methods We compared the clinical presentation and the outcome of patients with iTTP with coexistent SLE (SLE-iTTP) to an age-sex matched cohort of patients with idiopathic iTTP without SLE.Results During the study period, 1409 patients with iTTP were recruited in our registry. Of these, 79 (6%) had a prior or concurrent diagnosis of SLE at the time of iTTP diagnosis, and 437 (31%) had detectable ANAs without other clinical features of SLE. When compared with idiopathic iTTP, patients with SLE-iTTP had more severe renal involvement, and cardiac involvement was more prevalent, whereas central nervous system involvement was less common. Patients with SLE-iTTP received more immunosuppressive agents. There was no difference in response categories during the acute phase. During follow-up, SLE-iTTP had superior ADAMTS13 relapse-free survival than idiopathic iTTP. Among patients with ANAs without other clinical features of SLE, 33 (8%) were diagnosed with SLE 27 months (IQR: 7–65 months) following iTTP diagnosis; 32 additional patients (7%) developed another systemic autoimmune disease. No patient from the idiopathic iTTP group developed clinical SLE during follow-up.Conclusion Patients with iTTP are prone to develop autoimmune features, and patients with SLE-iTTP have distinct clinical features and outcome. Relapse-free survival seems better in patients with SLE-iTTP, underscoring the need for tailored management strategies in this population, including a specific follow-up to assess early features suggestive of SLE.Trial registration number NCT00426686.https://lupus.bmj.com/content/12/2/e001691.full |
| spellingShingle | David Ribes Nihal Martis Jehane Fadlallah Ygal Benhamou Veronique Le Guern Virginie Rieu Gabriel Choukroun Raïda Bouzid Agnès Veyradier Paul Coppo Bérangère S Joly Júlia Weisinger François Provôt Pascale Poullin Yahsou Delmas Sylvain Chantepie Manon Marie Ranta Dana Immune-mediated thrombotic thrombocytopenic purpura with systemic lupus erythematosus: clinical features and outcome Lupus Science and Medicine |
| title | Immune-mediated thrombotic thrombocytopenic purpura with systemic lupus erythematosus: clinical features and outcome |
| title_full | Immune-mediated thrombotic thrombocytopenic purpura with systemic lupus erythematosus: clinical features and outcome |
| title_fullStr | Immune-mediated thrombotic thrombocytopenic purpura with systemic lupus erythematosus: clinical features and outcome |
| title_full_unstemmed | Immune-mediated thrombotic thrombocytopenic purpura with systemic lupus erythematosus: clinical features and outcome |
| title_short | Immune-mediated thrombotic thrombocytopenic purpura with systemic lupus erythematosus: clinical features and outcome |
| title_sort | immune mediated thrombotic thrombocytopenic purpura with systemic lupus erythematosus clinical features and outcome |
| url | https://lupus.bmj.com/content/12/2/e001691.full |
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