{
  "abstract": "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.",
  "authors": [
    {
      "affiliations": [
        "Centre de Référence des Microangiopathies Thrombotiques et Service d’hématologie, Hôpital Saint Antoine, Assistance Publique-Hôpitaux de Paris, Sorbonne Université, Paris, France"
      ],
      "name": "Júlia Weisinger"
    },
    {
      "affiliations": [
        "Centre de Référence des Microangiopathies Thrombotiques et Service d’hématologie, Hôpital Saint Antoine, Assistance Publique-Hôpitaux de Paris, Sorbonne Université, Paris, France",
        "INSERM UMR-S 1138, Centre de Recherche des Cordeliers, Université Paris Cité, Sorbonne Université, Paris, France"
      ],
      "name": "Raïda Bouzid"
    },
    {
      "affiliations": [
        "Service d’Immunologie Clinique, Hôpital Saint-Louis, APHP, Paris, France"
      ],
      "name": "Jehane Fadlallah"
    },
    {
      "affiliations": [
        "Service de Néphrologie, Hôpital Albert Calmette, Lille, France"
      ],
      "name": "François Provôt"
    },
    {
      "affiliations": [
        "Service d’Hémaphérèse, Hôpital de La Conception, CHU de Marseille, Marseille, France"
      ],
      "name": "Pascale Poullin"
    },
    {
      "affiliations": [
        "Unité d’Hémaphérèse, Service de Médecine Interne, Hôpital Cochin, Paris, France"
      ],
      "name": "Véronique Le Guern"
    },
    {
      "affiliations": [
        "INSERM U1297, Department of Nephrology and Organ Transplantation, Referral Centre for Rare Kidney Diseases, University Hospital of Toulouse, Toulouse, France"
      ],
      "name": "David Ribes"
    },
    {
      "affiliations": [
        "Service de Médecine Interne et d’Immunologie Clinique, Hopital de Nice, Nice, France"
      ],
      "name": "Nihal Martis"
    },
    {
      "affiliations": [
        "Service de Néphrologie, CHU Bordeaux, Bordeaux, France"
      ],
      "name": "Yahsou Delmas"
    },
    {
      "affiliations": [
        "Institut d’Hématologie de Basse Normandie, Centre Hospitalier Universitaire, Caen, France"
      ],
      "name": "Sylvain Chantepie"
    },
    {
      "affiliations": [
        "Service de Médecine Interne, CHU de Clermont-Ferrand, Clermont-Ferrand, France"
      ],
      "name": "Virginie Rieu"
    },
    {
      "affiliations": [
        "Service de Médecine Interne, CHU Charles Nicolle, Rouen, France"
      ],
      "name": "Ygal Benhamou"
    },
    {
      "affiliations": [
        "Service de Néphrologie Dialyse et Transplantation, CHU d’Amiens et UPJV, Amiens, France"
      ],
      "name": "Gabriel Choukroun"
    },
    {
      "affiliations": [
        "Service de Médecine Interne, Hôpital Edouard Herriot, Hospices Civils de Lyon, Lyon, France"
      ],
      "name": "Manon Marie"
    },
    {
      "affiliations": [
        "Service d’Hématologie, Centre Hospitalier Universitaire de Nancy, Nancy, France"
      ],
      "name": "Ranta Dana"
    },
    {
      "affiliations": [
        "Centre de Référence des Microangiopathies Thrombotiques et Service d’hématologie, Hôpital Saint Antoine, Assistance Publique-Hôpitaux de Paris, Sorbonne Université, Paris, France",
        "Service d’Hématologie Biologique, Hôpital Lariboisière, AP-HP Nord, Université Paris Cité, Paris, France"
      ],
      "name": "Agnès Veyradier"
    },
    {
      "affiliations": [
        "Centre de Référence des Microangiopathies Thrombotiques et Service d’hématologie, Hôpital Saint Antoine, Assistance Publique-Hôpitaux de Paris, Sorbonne Université, Paris, France",
        "Service d’Hématologie Biologique, Hôpital Lariboisière, AP-HP Nord, Université Paris Cité, Paris, France"
      ],
      "name": "Bérangère S Joly"
    },
    {
      "affiliations": [
        "Centre de Référence des Microangiopathies Thrombotiques et Service d’hématologie, Hôpital Saint Antoine, Assistance Publique-Hôpitaux de Paris, Sorbonne Université, Paris, France",
        "INSERM UMR-S 1138, Centre de Recherche des Cordeliers, Université Paris Cité, Sorbonne Université, Paris, France"
      ],
      "name": "Paul Coppo"
    }
  ],
  "title": "Immune-mediated thrombotic thrombocytopenic purpura with systemic lupus erythematosus: clinical features and outcome",
  "uid": "c3bf9b1a-9b07-5bea-b722-0c2f8cea01a1"
}