{
  "abstract": "Objective To compare patients with SLE associated with either Evans syndrome (ES) or an isolated autoimmune cytopenia (immune thrombocytopenia (ITP) or autoimmune haemolytic anaemia (AIHA)).Methods Multicentre retrospective study including patients with SLE presenting with ITP, AIHA or ES of clinical significance (ie, requiring therapeutic intervention according to European Alliance of Associations for Rheumatology guidelines or clinician discretion). Clinical, laboratory and outcome data were compared between patients with ES and those with ITP or AIHA. Severe SLE flares were defined as flares with SLE Disease Activity Index ≥10.Results Among 95 patients with SLE included, 30 had ES, 43 ITP and 22 AIHA. The number of severe SLE flares per patient was higher in ES than in ITP (1.3 vs 0.4, p=0.0004) and patients with AIHA (0.4, p=0.006). Patients with ES had a higher incidence rate ratio (IRR) of severe SLE flares (IRR =3.03; 95% CI 1.50 to 6.41), which remained significant in inverse probability of treatment weighting analyses. At last follow-up, patients with ES presented higher rates of renal (36.7% vs 9.3%, p=0.007) and neurological (36.7% vs 11.6%, p=0.019) involvement than patients with ITP. Severe infections were more frequent in patients with ES than ITP (47% vs 23%, p=0.045), with a higher mean number of severe infections per patient (1.2 vs 0.5, p=0.04).Conclusion In patients with SLE, ES is associated with an increased risk of severe flares than isolated autoimmune cytopenia. These findings were consistent after adjustment for baseline imbalances, supporting ES as a high-risk SLE phenotype.",
  "authors": [
    {
      "affiliations": [
        "Hôpital de la Croix Rousse,Service de Médecine Interne, Hospices Civils de Lyon, Lyon, France",
        "UMR CNRS 5305 Laboratoire de Biologie Tissulaire et Ingénierie Thérapeutique, Université Claude Bernard Lyon 1, Lyon, France"
      ],
      "name": "Thomas El-Jammal"
    },
    {
      "affiliations": [
        "Hôpital de la Croix Rousse,Service de Médecine Interne, Hospices Civils de Lyon, Lyon, France"
      ],
      "name": "Anne Fabre-Barthez"
    },
    {
      "affiliations": [
        "Centre Hospitalier Lyon Sud, Service de Médecine Interne, Hospices Civils de Lyon, Lyon, France"
      ],
      "name": "Nicolas Martinet"
    },
    {
      "affiliations": [
        "Centre Hospitalier Lyon Sud, Service de Médecine Interne, Hospices Civils de Lyon, Lyon, France"
      ],
      "name": "Mickaël Roussotte"
    },
    {
      "affiliations": [
        "Hôpital Edouard Herriot, Service de Médecine Interne, Hospices Civils de Lyon, Lyon, France"
      ],
      "name": "Arnaud Hot"
    },
    {
      "affiliations": [
        "Service de Médecine Interne, Annecy Genevois Hospital, Épagny Metz-Tessy, France"
      ],
      "name": "Antoine Baudet"
    },
    {
      "affiliations": [
        "Centre Hospitalier Lyon Sud, Service de Médecine Interne, Hospices Civils de Lyon, Lyon, France"
      ],
      "name": "Quitterie Reynaud"
    },
    {
      "affiliations": [
        "Centre Hospitalier Lyon Sud, Service de Médecine Interne, Hospices Civils de Lyon, Lyon, France"
      ],
      "name": "Isabelle Durieu"
    },
    {
      "affiliations": [
        "Service de Médecine Interne, Centre Hospitalier Saint Joseph Saint Luc, Lyon, France"
      ],
      "name": "Laurent Pérard"
    },
    {
      "affiliations": [
        "Hôpital Edouard Herriot, Service de Médecine Interne, Hospices Civils de Lyon, Lyon, France"
      ],
      "name": "Thomas Barba"
    },
    {
      "affiliations": [
        "Hôpital de la Croix Rousse,Service de Médecine Interne, Hospices Civils de Lyon, Lyon, France",
        "Research On Healthcare Performance, INSERM U1290, Lyon, France"
      ],
      "name": "Pascal Sève"
    }
  ],
  "title": "Comparison of Evans syndrome and isolated autoimmune cytopenias in SLE: a retrospective study",
  "uid": "9f2a0c7c-ccbd-5794-9030-a3ecbd45f7c6"
}