{
  "abstract": "Background Although colchicine is the mainstay of familial Mediterranean fever (FMF) treatment, 5–10% of patients are considered to have colchicine resistance (CR). However, there is no globally agreed CR definition or indications for biological disease-modifying anti-rheumatic drugs (bDMARDs).Methods A survey on ‘Biologics in Monogenic Autoinflammatory Diseases’, part of the ‘Clinical Practice Strategies’ (CLiPS) initiative, was conducted by a JIR cohort-initiated eCOST network among expert participants worldwide. Our primary aim was to provide a flowchart reflecting the different CR definitions and present data regarding bDMARD indications. The secondary aim was to determine how specific biases influence clinical approaches. We analysed the CliPS according to the experience levels of physicians, country-specific FMF prevalence, countries’ gross domestic product, bDMARD availability and reimbursement policies of the countries.Results A total of 223 responses from 46 countries were included in the study. Almost half of the respondents (73/160, 45.6%) indicated that three to four attacks within the preceding 6 months were necessary for their CR definition. The most frequently used acute-phase reactant was C-reactive protein (157/164, 95.7%). Almost three-fourths of the respondents (74%, n=165) considered that supplementary factors, including complications of FMF, attack severity, elevated activity scores, patient-reported outcome and quality of life scales, influenced their CR definition.Conclusion We present a novel flowchart describing physicians’ general attitudes and unique findings regarding management strategies for colchicine-resistant FMF and shifting trends influenced by epidemiological and socioeconomic factors.",
  "authors": [
    {
      "affiliations": [
        "Department of Pediatric Rheumatology, Istanbul University-Cerrahpasa Cerrahpasa Faculty of Medicine, Fatih, Turkey",
        "Department of Pediatric Rheumatology, Istanbul Medeniyet University Faculty of Medicine, Üsküdar, Turkey"
      ],
      "name": "Fatih Haslak"
    },
    {
      "affiliations": [
        "Pediatric Rheumatology, University of Health Sciences, Gulhane Training and Research Hospital, Ankara, Turkey"
      ],
      "name": "Nimet Oner"
    },
    {
      "affiliations": [
        "General Pediatrics, Hospital Centre Versailles, Le Chesnay, France",
        "Internal Medicine, AP-HP, Tenon Hospital, Paris, France"
      ],
      "name": "Inès Elhani"
    },
    {
      "affiliations": [
        "Clinic for Paediatric and Adolescent Rheumatology, St. Josef-Stift Hospital, Northwest German Center for Rheumatology, Sendenhorst, Germany"
      ],
      "name": "Tanja Hinze"
    },
    {
      "affiliations": [
        "Unaffiliated Researcher, Participant of a Short-Term Scientific Mission at COST Action CA21168, Berlin, Germany"
      ],
      "name": "Anna Mamutova"
    },
    {
      "affiliations": [
        "Internal Medicine, Hospital of interior security, La Marsa, Tunisia, University Tunis El Manar, Faculté de médecine de Tunis, La Marsa, Tunisia"
      ],
      "name": "Rim Bourguiba"
    },
    {
      "affiliations": [
        "Department of Pediatrics, Division of Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey"
      ],
      "name": "Muserref Kasap Cuceoglu"
    },
    {
      "affiliations": [
        "Department of Statistics, Athens University of Economics and Business, Athens, Greece",
        "Department of Public and One Health, University of Thessaly, Karditsa, Greece"
      ],
      "name": "Konstantinos Pateras"
    },
    {
      "affiliations": [
        "Pediatric Rheumatology, Ruth Rappaport Children’s Hospital, Haifa, Israel"
      ],
      "name": "Yonatan Butbul Aviel"
    },
    {
      "affiliations": [
        "Internal Medicine, Sorbonne Université, AP- HP, Centre de référence des maladies autoinflammatoires et des amyloses (CEREMAIA), Hôpital Tenon, Paris, France"
      ],
      "name": "Marion Delplanque"
    },
    {
      "affiliations": [
        "UOC Reumatologia e malattie autoinfiammatorie, IRCCS Istituto Giannina Gaslini, Genoa, Italy"
      ],
      "name": "Roberta Caorsi"
    },
    {
      "affiliations": [
        "Department of Paediatrics, University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia"
      ],
      "name": "Mario Šestan"
    },
    {
      "affiliations": [
        "Internal Medicine, Hôpital du Sacré-Coeur de Montréal, Montreal, Quebec, Canada"
      ],
      "name": "Stéphanie Ducharme Bénard"
    },
    {
      "affiliations": [
        "Department of Paediatrics, Medical University Innsbruck, Innsbruck, Tyrol, Austria",
        "Faculty of Medicine and Dentistry, Danube Private University, Krems an der Donau, Lower Austria, Austria"
      ],
      "name": "Jürgen Brunner"
    },
    {
      "affiliations": [
        "Infectious Diseases and General Internal Medicine, University Hospital of Liège GIGA-Inflammation, Infection & Immunity | University of Liège, Liège, Belgium"
      ],
      "name": "Majdouline El Moussaoui"
    },
    {
      "affiliations": [
        "Institute of Immunobiology and Human Genetics, Faculty of Medicine, Ss. Cyril and Methodius University in Skopje, 1000 Skopje, Skopje, North Macedonia"
      ],
      "name": "Meri Kirijas"
    },
    {
      "affiliations": [
        "Pediatric Centre, Semmelweis University of Medicine, Budapest, Hungary"
      ],
      "name": "Tamas Constantin"
    },
    {
      "affiliations": [
        "Pediatric Rheumatology Department, Hospital Sant Joan de Déu, Barcelona, Spain"
      ],
      "name": "Sonia Carriquí Arenas"
    },
    {
      "affiliations": [
        "Nephrology department, University of Algiers, Faculty of medicine, Mohamed lamine debaghine Hospital, Algiers, Algeria"
      ],
      "name": "Ghalia Khellaf"
    },
    {
      "affiliations": [
        "Pediatric Rheumatology, Istanbul University Istanbul Faculty of Medicine, Istanbul, Turkey"
      ],
      "name": "Vafa Guliyeva"
    },
    {
      "affiliations": [
        "Pediatric Rheumatology Unit, Schneider Children’s Medical Center of Israel, Petah Tekvah, Israel"
      ],
      "name": "Naiera Assalia"
    },
    {
      "affiliations": [
        "Pediatric Rheumatology and Immunology, University Medicine, Albert-Schweitzer-Campus 1, Munster, Germany"
      ],
      "name": "Stefan Backes"
    },
    {
      "affiliations": [
        "Departments of Pediatric Rheumatology, Umraniye Training and Research Hospital, University of Health Sciences, Istanbul, Turkey"
      ],
      "name": "Betul Sozeri"
    },
    {
      "affiliations": [
        "Department of Paediatrics, Hôpital Riviera-Chablais, Rennaz, Switzerland",
        "Fondation Rhumatismes Enfants Suisse, Lausanne, Switzerland"
      ],
      "name": "Michaël Hofer"
    },
    {
      "affiliations": [
        "Pediatric Rheumatology, Istanbul University Istanbul Faculty of Medicine, Istanbul, Turkey"
      ],
      "name": "Nuray Ayaz"
    },
    {
      "affiliations": [
        "Pediatric Rheumatology, National Amyloidosis Centre, London, UK"
      ],
      "name": "Helen Lachmann"
    },
    {
      "affiliations": [
        "Department of paediatric Rheumatology and Immunology, University Children’s Hospital Muenster, Muenster, Germany"
      ],
      "name": "Helmut Wittkowski"
    },
    {
      "affiliations": [
        "Pediatrics, French Reference Center for Autoinflammatory Diseases and Amyloidosis, Versailles Hospital, Versailles, France"
      ],
      "name": "Véronique Hentgen"
    }
  ],
  "title": "Comparative analysis of global practices in the management of colchicine-resistant familial Mediterranean fever: a CliPS network analysis",
  "uid": "f0cb8713-8332-57f2-842c-46d1ae7e851a"
}