{
  "abstract": "Objective To investigate the concordance between organ involvement at diagnosis and relapse in granulomatosis with polyangiitis and factors associated with new disease features at relapse.Methods Data from a national database of newly diagnosed patients was analysed. Clinical features were recorded at diagnosis and relapse, grouped by organ system. ORs and HRs were used to assess associations between baseline features and first relapse. Factors independently associated with new organ involvement at relapse were identified using multivariable logistic regression.Results Among 795 patients (median follow-up 3.5 years), 394 (50%) relapsed; organ involvement at relapse was available for 376 patients. Relapses most often affected ear, nose and throat (ENT), lungs and kidneys. Organ involvement at diagnosis was associated with a higher likelihood of relapse in the same organ: eyes (OR 6.69), lungs (OR 3.35), kidneys (OR 3.58), nervous system (OR 2.90), and mucocutaneous (OR 4.53). Major manifestations associated with a higher likelihood of recurrence were scleritis, pachymeningitis, subglottic stenosis and worsening renal function. For 56% of patients, the first relapse affected only the initially involved organs. Of the 165 patients with new organ manifestations, these were rarely isolated (n=34) and usually occurred alongside involvement of at least one previously affected organ (n=131). In multivariable analysis, systemic, ENT and lung manifestations at diagnosis were associated with a lower risk of new organ disease at relapse.Conclusion Although new features can still emerge, organ involvement at diagnosis is associated with a higher likelihood of relapse in the same organ.",
  "authors": [
    {
      "affiliations": [
        "Division of rheumatology, Geneva University Hospitals and University of Geneva, Geneva, Switzerland",
        "Department of Internal Medicine, Cochin Hospital, National Referral Center for Rare Systemic Autoimmune and Autoinflammatory Diseases of Ile de France, East and West, Assistance Publique - Hôpitaux de Paris, Paris, France"
      ],
      "name": "Michele Iudici"
    },
    {
      "affiliations": [
        "University of Montpellier, Montpellier, France",
        "Inserm U1046, CNRS UMR 9214, PhyMedExp, Montpellier, France"
      ],
      "name": "Zubeyir Salis"
    },
    {
      "affiliations": [
        "Department of Internal Medicine, Cochin Hospital, National Referral Center for Rare Systemic Autoimmune and Autoinflammatory Diseases of Ile de France, East and West, Assistance Publique - Hôpitaux de Paris, Paris, France"
      ],
      "name": "Pascal Cohen"
    },
    {
      "affiliations": [
        "Department of Internal Medicine, Nantes University Hospital, and Nantes Université, INSERM, Center for Research in Transplantation and Translational Immunology (CR2TI), UMR 1064, Nantes, France"
      ],
      "name": "Antoine Néel"
    },
    {
      "affiliations": [
        "Département de médecine interne, CHU Caen, Caen, France, Caen, France"
      ],
      "name": "Achille Aouba"
    },
    {
      "affiliations": [
        "Department of Internal Medicine, Dax Hospital, Dax, France"
      ],
      "name": "Francois Lifermann"
    },
    {
      "affiliations": [
        "Université Clermont Auvergne, CNRS, CHU Clermont-Ferrand, Institut Pascal, F-63000, Clermont-Ferrand, France"
      ],
      "name": "Marc Ruivard"
    },
    {
      "affiliations": [
        "Université Clermont Auvergne, CNRS, CHU Clermont-Ferrand, Institut Pascal, F-63000, Clermont-Ferrand, France"
      ],
      "name": "Olivier Aumaître"
    },
    {
      "affiliations": [
        "Department of Internal Medicine and Clinical Immunology, Referral Center for Rare Systemic Autoimmune and Autoinflammatory Diseases, Dijon-Bourgogne University Hospital, University EPE Bourgogne Europe, Dijon, France"
      ],
      "name": "Bernard Bonnotte"
    },
    {
      "affiliations": [
        "Department of Internal Medicine and Clinical Immunology, Referral Center for Rare Systemic Autoimmune and Autoinflammatory Diseases, Dijon-Bourgogne University Hospital, University EPE Bourgogne Europe, Dijon, France"
      ],
      "name": "Maxime Samson"
    },
    {
      "affiliations": [
        "Internal Medicine, UNEOS Hospitals, Metz-Ventoux, Metz, France"
      ],
      "name": "Francois Maurier"
    },
    {
      "affiliations": [
        "Internal Medicine and Clinical Immunology, University of Rennes 1, Rennes, France"
      ],
      "name": "Thomas Le Gallou"
    },
    {
      "affiliations": [
        "Department of Internal Medicine and Clinical immunology, National Referral Centre for Rare Systemic Auto-immune and auto-inflammatory Diseases, Univ. Lille, Inserm, CHU Lille, U1286 – INFINITE, University of Lille, Lille, France"
      ],
      "name": "Eric Hachulla"
    },
    {
      "affiliations": [
        "Nephrology, European Hospital Georges Pompidou, AP-HP, University Paris-Cité, Paris, France"
      ],
      "name": "Alexandre Karras"
    },
    {
      "affiliations": [
        "National Referral Center for Rare Pulmonary Diseases, Louis-Pradel Hospital, Claude-Bernard University Lyon 1, Lyon, France"
      ],
      "name": "Vincent Cottin"
    },
    {
      "affiliations": [
        "APHM, CHU de la Conception, Nephrology, Aix-Marseille Univ, C2VN, INSERM, INRAE, Marseille, France"
      ],
      "name": "Noemie Jourde-Chiche"
    },
    {
      "affiliations": [
        "Internal Medicine, Haut-Lévêque Hospital, University of Bordeaux, Bordeaux, France"
      ],
      "name": "Jean-François Viallard"
    },
    {
      "affiliations": [
        "Department of Internal Medicine, Niort Hospital, Niort, France"
      ],
      "name": "Amandine Perier"
    },
    {
      "affiliations": [
        "Internal Medicine, Bretagne - Atlantique Hospital, Vannes, France"
      ],
      "name": "Pascal Godmer"
    },
    {
      "affiliations": [
        "Nephrology and Internal Medicine, Valenciennes Hospital, Valenciennes, France"
      ],
      "name": "Thomas Quéméneur"
    },
    {
      "affiliations": [
        "Internal Medicine, La Cavale Blanche University Hospital, Brest, France"
      ],
      "name": "Claire de Moreuil"
    },
    {
      "affiliations": [
        "Department of Internal Medicine, Cochin Hospital, National Referral Center for Rare Systemic Autoimmune and Autoinflammatory Diseases of Ile de France, East and West, Assistance Publique - Hôpitaux de Paris, Paris, France"
      ],
      "name": "Loïc Guillevin"
    },
    {
      "affiliations": [
        "Department of Internal Medicine, Cochin Hospital, National Referral Center for Rare Systemic Autoimmune and Autoinflammatory Diseases of Ile de France, East and West, Assistance Publique - Hôpitaux de Paris, Paris, France"
      ],
      "name": "Benjamin Terrier"
    },
    {
      "affiliations": [
        "Department of Internal Medicine, Cochin Hospital, National Referral Center for Rare Systemic Autoimmune and Autoinflammatory Diseases of Ile de France, East and West, Assistance Publique - Hôpitaux de Paris, Paris, France"
      ],
      "name": "Xavier Puéchal"
    },
    {
      "affiliations": [],
      "name": "French Vasculitis Study Group"
    }
  ],
  "title": "Concordance between presenting features and relapse in granulomatosis with polyangiitis: implications for risk assessment and counselling",
  "uid": "5157697b-4399-554c-9a49-bb95ba913df6"
}