Article Dans Une Revue Lupus science & medicine Année : 2025

Immune-mediated thrombotic thrombocytopenic purpura with systemic lupus erythematosus: clinical features and outcome

Júlia Weisinger
Raïda Bouzid
  • Fonction : Auteur
Jehane Fadlallah
  • Fonction : Auteur
François Provôt
  • Fonction : Auteur
Pascale Poullin
  • Fonction : Auteur
Véronique Le Guern
  • Fonction : Auteur
David Ribes
  • Fonction : Auteur
Nihal Martis
  • Fonction : Auteur
Yahsou Delmas
  • Fonction : Auteur
Sylvain Chantepie
  • Fonction : Auteur
Ygal Benhamou
  • Fonction : Auteur
Manon Marie
  • Fonction : Auteur
Ranta Dana
  • Fonction : Auteur
Agnès Veyradier
  • Fonction : Auteur
Bérangère Joly
Paul Coppo
  • Fonction : Auteur

Résumé

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 .

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Dates et versions

hal-05202608 , version 1 (07-08-2025)

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Júlia Weisinger, Raïda Bouzid, Jehane Fadlallah, François Provôt, Pascale Poullin, et al.. Immune-mediated thrombotic thrombocytopenic purpura with systemic lupus erythematosus: clinical features and outcome. Lupus science & medicine, 2025, 12 (2), pp.e001691. ⟨10.1136/lupus-2025-001691⟩. ⟨hal-05202608⟩
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