{
  "abstract": "Background Mixed connective tissue disease (MCTD) has long been debated as an early nonspecific phase/symptom of differentiated connective tissue diseases (dCTD), similarly to interstitial pneumonia with autoimmune features (IPAF) and very early diagnosis of systemic sclerosis (SSc) (VEDOSS).Objective We aimed to evaluate the predictive value of IPAF, VEDOSS and dCTD classification criteria variables in MCTD patients.Methods We conducted an observational study within the French MCTD cohort. IPAF, VEDOSS and current dCTD classification criteria were used to classify patients.Results Three hundred and twenty-four MCTD patients were included and followed for 8 (3.3–13) years. Among them, 111 (34.3%) progressed into a dCTD, that is, 50 (15.4%) SSc, 40 (12.3%) systemic lupus erythematosus (SLE) and 11 (3.4%) Sjögren’s disease. At diagnosis, 38 (11.7%) patients fulfilled IPAF criteria, among which 15 (39.5%) progressed into a dCTD (vs 75 (26.2%) in patients who did not fulfil IPAF criteria; p=0.09). At diagnosis, 293 (90.4%) patients fulfilled VEDOSS criteria but did not progress significantly more frequently to SSc than MCTD patients without VEDOSS criteria (46 (15.7%) vs 4 (12.9%); p=0.8). At baseline, SSc classification criteria did not predict evolution toward SSc, whereas antiphospholipid antibodies and low C3 and/or C4 were predictive of an evolution toward SLE (p=0.01 and p=0.04, respectively).Conclusion At MCTD diagnosis, fulfilment of IPAF and/or VEDOSS criteria was not predictive of evolution toward SSc, whereas antiphospholipid antibodies and low C3 and/or C4 were predictive of an evolution toward SLE. This suggests that MCTD patients should be excluded from IPAF and VEDOSS.",
  "authors": [
    {
      "affiliations": [
        "Department of Internal Medicine, Centre de Référence Maladies Auto-Immunes et Systémiques Rares d’Ile de France, AP-HP.Centre, Université Paris Cité, Paris, France"
      ],
      "name": "Kevin Chevalier"
    },
    {
      "affiliations": [
        "Department of Internal Medicine, CHRU de Tours, Tours, France"
      ],
      "name": "Benjamin Thoreau"
    },
    {
      "affiliations": [
        "Department of Internal Medicine, Hôpital Henri Mondor, Créteil, France"
      ],
      "name": "Marc Michel"
    },
    {
      "affiliations": [
        "Department of Internal Medicine, Hôpital Henri Mondor, Créteil, France"
      ],
      "name": "Bertrand Godeau"
    },
    {
      "affiliations": [
        "Service de Médecine Interne, Centre Hospitalier Universitaire de Nantes, Nantes, France"
      ],
      "name": "Christian Agard"
    },
    {
      "affiliations": [
        "Hôpital Bichat Claude-Bernard, Paris, France"
      ],
      "name": "Thomas Papo"
    },
    {
      "affiliations": [
        "Hôpital Bichat Claude-Bernard, Paris, France"
      ],
      "name": "Karim Sacre"
    },
    {
      "affiliations": [
        "Pediatric Hematology-Immunology and Rheumatology, Hopital universitaire Necker-Enfants malades, Paris, France"
      ],
      "name": "Brigitte Bader-Meunier"
    },
    {
      "affiliations": [
        "Rheumatology, Hopital Bicetre, Le Kremlin-Bicêtre, France"
      ],
      "name": "Raphaele Seror"
    },
    {
      "affiliations": [
        "Rheumatology, Hopital Bicetre, Le Kremlin-Bicêtre, France"
      ],
      "name": "Xavier Mariette"
    },
    {
      "affiliations": [
        "Service de médecine interne et immunologie clinique, Hopital Pitie-Salpetriere, Paris, France"
      ],
      "name": "Patrice Cacoub"
    },
    {
      "affiliations": [
        "CHU de Rouen, Rouen, France"
      ],
      "name": "Ygal Benhamou"
    },
    {
      "affiliations": [
        "CHU de Rouen, Rouen, France"
      ],
      "name": "Hervé Levesque"
    },
    {
      "affiliations": [
        "Internal medicine, Hôpital Bicêtre, AP-HP, Université Paris Saclay, Kremlin-Bicêtre, France"
      ],
      "name": "Cécile Goujard"
    },
    {
      "affiliations": [
        "Internal medicine, Hôpital Bicêtre, AP-HP, Université Paris Saclay, Kremlin-Bicêtre, France"
      ],
      "name": "Olivier Lambotte"
    },
    {
      "affiliations": [
        "Internal Medicine and Clinical Immunology department, Referral Center for Rare Systemic Autoimmune and Autoinflammatory Diseases, University Hospital Centre Dijon Bourgogne, Dijon, France"
      ],
      "name": "Bernard Bonnotte"
    },
    {
      "affiliations": [
        "Internal Medicine and Clinical Immunology department, Referral Center for Rare Systemic Autoimmune and Autoinflammatory Diseases, University Hospital Centre Dijon Bourgogne, Dijon, France"
      ],
      "name": "Maxime Samson"
    },
    {
      "affiliations": [
        "Department of Internal Medicine, Hospital Foch, Suresnes, France"
      ],
      "name": "Félix Ackermann"
    },
    {
      "affiliations": [
        "Internal Medicine, CHU Amiens-Picardie, Amiens, France"
      ],
      "name": "Jean Schmidt"
    },
    {
      "affiliations": [
        "Internal Medicine, CHU Amiens-Picardie, Amiens, France"
      ],
      "name": "Pierre Duhaut"
    },
    {
      "affiliations": [
        "Service de Rhumatologie Pédiatrique, Centre de Référence des Maladies Auto-Inflammatoires de l’enfant, Hôpital Bicêtre, Le Kremlin-Bicêtre, France"
      ],
      "name": "Isabelle Koné-Paut"
    },
    {
      "affiliations": [
        "Department of Internal Medicine, Hopital Ambroise-Pare, Boulogne-Billancourt, France"
      ],
      "name": "Jean-Emmanuel Kahn"
    },
    {
      "affiliations": [
        "Department of Internal Medicine, Hopital Ambroise-Pare, Boulogne-Billancourt, France"
      ],
      "name": "Thomas Hanslik"
    },
    {
      "affiliations": [
        "Department of Internal Medicine, Centre de Référence Maladies Auto-Immunes et Systémiques Rares d’Ile de France, AP-HP.Centre, Université Paris Cité, Paris, France"
      ],
      "name": "Nathalie Costedoat-Chalumeau"
    },
    {
      "affiliations": [
        "Department of Internal Medicine, Centre de Référence Maladies Auto-Immunes et Systémiques Rares d’Ile de France, AP-HP.Centre, Université Paris Cité, Paris, France"
      ],
      "name": "Benjamin Terrier"
    },
    {
      "affiliations": [
        "Department of Internal Medicine, Centre de Référence Maladies Auto-Immunes et Systémiques Rares d’Ile de France, AP-HP.Centre, Université Paris Cité, Paris, France"
      ],
      "name": "Alexis Regent"
    },
    {
      "affiliations": [
        "Department of Internal Medicine, Centre de Référence Maladies Auto-Immunes et Systémiques Rares d’Ile de France, AP-HP.Centre, Université Paris Cité, Paris, France"
      ],
      "name": "Bertrand Dunogue"
    },
    {
      "affiliations": [
        "Department of Internal Medicine, Centre de Référence Maladies Auto-Immunes et Systémiques Rares d’Ile de France, AP-HP.Centre, Université Paris Cité, Paris, France"
      ],
      "name": "Pascal Cohen"
    },
    {
      "affiliations": [
        "Department of Internal Medicine, Centre de Référence Maladies Auto-Immunes et Systémiques Rares d’Ile de France, AP-HP.Centre, Université Paris Cité, Paris, France"
      ],
      "name": "Véronique Le Guern"
    },
    {
      "affiliations": [
        "Internal medicine, Lille University Hospital, Lille, France, Lille, France"
      ],
      "name": "Eric Hachulla"
    },
    {
      "affiliations": [
        "Department of Internal Medicine, Centre de Référence Maladies Auto-Immunes et Systémiques Rares d’Ile de France, AP-HP.Centre, Université Paris Cité, Paris, France"
      ],
      "name": "Benjamin Chaigne"
    },
    {
      "affiliations": [
        "Department of Internal Medicine, Centre de Référence Maladies Auto-Immunes et Systémiques Rares d’Ile de France, AP-HP.Centre, Université Paris Cité, Paris, France"
      ],
      "name": "Luc Mouthon"
    }
  ],
  "title": "Unravelling IPAF, VEDOSS and connective tissue diseases classifications through the mixed connective tissue disease spectrum",
  "uid": "aa5469c2-21d4-573f-929c-d99d70b2c5b9"
}