{
  "abstract": "Introduction Patients with adult refractory coeliac disease type 1 (RCD1) are defined as those with persistent symptoms despite strict adherence to a gluten-free diet for at least 12 months and ongoing villous atrophy (in the absence of a significant aberrant intraepithelial lymphocyte population). Budesonide therapy has been described in small single centre studies. However, data supporting its efficacy and defining its risk profile in RCD1 remains limited. We present the 1st multi-centre study by the European Society for the Study of Coeliac Disease (ESsCD) to assess this.Methods All patients with RCD1 treated with budesonide from 11 hospitals (United Kingdom, Germany, Italy, Netherlands, France, Republic of Ireland, Malta and Spain) over a 23 year period were assessed for the effects of treatment with steroids. The steroid sparing agents used and side effects were also analysed.Results In total, 186 patients were treated with budesonide for refractory coeliac disease ((72.0% female, 41.9 years (IQR 29.6-53.0 years) at coeliac disease diagnosis). Patients were started on budesonide, a median of 7.0 years (IQR:3.2-16.0 years) after diagnosis. On budesonide, 50.0% of patients reported complete resolution of their symptoms, 31.2% some improvement, 17.7% no benefit to their symptoms and 1.1% a worsening of their symptoms. Of those who had a repeat gastroscopy on budesonide (n=134), 52.2% exhibited a histological improvement. A clinical response to budesonide was associated with a higher likelihood of histological improvement (OR: 9.4 (95% CI: 2.6-33.7). There was no association between age (p=0.158) or sex (p=0.787) and clinical response to budesonide.Side effects were reported by 5.9% of patients and adrenal insufficiency identified in 2 of 12 patients tested.Following initiation of budesonide treatment, 39.8% of patients required no further treatment, 19.9% required long term budesonide and 32.1% of patients required restarting or uptitrating of their budesonide treatment due to a return of symptoms. In those requiring further treatments, the most frequently trialled steroid sparing agent was azathioprine and 47.1% of patients who started it continued therapy long term.Conclusion We present the first real world multi-centre international study demonstrating that over 80% of patients symptomatically benefited from budesonide. However, of those, only half had histological remission and over 30% relapsed on withdrawal of budesonide. This study provides the evidence that budesonide is the first line treatment of choice for RCD I.",
  "authors": [
    {
      "affiliations": [
        "Academic Unit of Gastroenterology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, United Kingdom",
        "Division of Clinical Medicine, University of Sheffield, Sheffield, United Kingdom"
      ],
      "name": "Suneil Raju"
    },
    {
      "affiliations": [
        "Academic Unit of Gastroenterology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, United Kingdom",
        "Division of Clinical Medicine, University of Sheffield, Sheffield, United Kingdom"
      ],
      "name": "Flora Kokwaro"
    },
    {
      "affiliations": [
        "Academic Unit of Gastroenterology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, United Kingdom",
        "Division of Clinical Medicine, University of Sheffield, Sheffield, United Kingdom"
      ],
      "name": "Nicoletta Nandi"
    },
    {
      "affiliations": [
        "Academic Unit of Gastroenterology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, United Kingdom",
        "Department of Translational Medicine, University of Ferrara, Ferrara, Italy"
      ],
      "name": "Francesca Manza"
    },
    {
      "affiliations": [
        "Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy",
        "Medizinische Klinik Für Gastroenterologie, Charité—Universitätsmedizi, Berlin, Germany"
      ],
      "name": "Matilde Topa"
    },
    {
      "affiliations": [
        "Department of Surgery, Oncology, Gastroenterology, University of Padua, Padua, Italy",
        "Gastroenterology Unit, Azienda Ospedale-Università Padova, Padua, Italy"
      ],
      "name": "Cristina Caranfil"
    },
    {
      "affiliations": [
        "Department of Internal Medicine and Therapeutics, University of Pavia, Pavia, Italy",
        "Istituti Clinici Scientifici Maugeri IRCCS, Gastroenterology Unit of Pavia Institute, Pavia, Italy"
      ],
      "name": "Annalisa Schiepatti"
    },
    {
      "affiliations": [
        "Department of Gastroenterology and Endoscopy, CELAC Network, AP-HP Centre, Hôpital Européen Georges Pompidou, Université de Paris, Cité and Institut National du Cancer, Paris, France"
      ],
      "name": "Sherine Khater"
    },
    {
      "affiliations": [
        "Department of Gastroenterology, Galway University Hospital, Galway, Ireland"
      ],
      "name": "Gillian Madders"
    },
    {
      "affiliations": [
        "Department of Internal Medicine and Therapeutics, University of Pavia, Pavia, Italy",
        "Istituti Clinici Scientifici Maugeri IRCCS, Gastroenterology Unit of Pavia Institute, Pavia, Italy"
      ],
      "name": "Chiara Scarcella"
    },
    {
      "affiliations": [
        "Department of Internal Medicine and Therapeutics, University of Pavia, Pavia, Italy",
        "Istituti Clinici Scientifici Maugeri IRCCS, Gastroenterology Unit of Pavia Institute, Pavia, Italy"
      ],
      "name": "Giusy Memoli"
    },
    {
      "affiliations": [
        "Academic Unit of Gastroenterology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, United Kingdom",
        "Division of Clinical Medicine, University of Sheffield, Sheffield, United Kingdom"
      ],
      "name": "Manjeevan Singh"
    },
    {
      "affiliations": [
        "Academic Unit of Gastroenterology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, United Kingdom",
        "Division of Clinical Medicine, University of Sheffield, Sheffield, United Kingdom"
      ],
      "name": "Alicia Raybould"
    },
    {
      "affiliations": [
        "Academic Unit of Gastroenterology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, United Kingdom",
        "Division of Clinical Medicine, University of Sheffield, Sheffield, United Kingdom"
      ],
      "name": "Rebecca Stone"
    },
    {
      "affiliations": [
        "Academic Unit of Gastroenterology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, United Kingdom",
        "Division of Clinical Medicine, University of Sheffield, Sheffield, United Kingdom"
      ],
      "name": "Liam MacManus"
    },
    {
      "affiliations": [
        "Academic Unit of Gastroenterology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, United Kingdom",
        "Division of Clinical Medicine, University of Sheffield, Sheffield, United Kingdom"
      ],
      "name": "Rosie Flack"
    },
    {
      "affiliations": [
        "Academic Unit of Gastroenterology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, United Kingdom",
        "Division of Clinical Medicine, University of Sheffield, Sheffield, United Kingdom"
      ],
      "name": "Grace Brownlow"
    },
    {
      "affiliations": [
        "Department of Gastroenterology and Endoscopy, CELAC Network, AP-HP Centre, Hôpital Européen Georges Pompidou, Université de Paris, Cité and Institut National du Cancer, Paris, France"
      ],
      "name": "Boris Rosenbaum"
    },
    {
      "affiliations": [
        "Department of Gastroenterology and Endoscopy, CELAC Network, AP-HP Centre, Hôpital Européen Georges Pompidou, Université de Paris, Cité and Institut National du Cancer, Paris, France"
      ],
      "name": "Alexandre Bai"
    },
    {
      "affiliations": [
        "Department of Gastroenterology, Hospital Universitari Mútua Terrassa, University of Barcelona, Barcelona, Spain",
        "Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas (CIBERehd), Instituto de Salud Carlos III, Madrid, Spain"
      ],
      "name": "Maria Esteve-Comas"
    },
    {
      "affiliations": [
        "Department of Gastroenterology, Hospital Universitari Mútua Terrassa, University of Barcelona, Barcelona, Spain",
        "Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas (CIBERehd), Instituto de Salud Carlos III, Madrid, Spain"
      ],
      "name": "Albert Cardona-Martin"
    },
    {
      "affiliations": [
        "Department of Gastroenterology, Galway University Hospital, Galway, Ireland"
      ],
      "name": "Valerie Byrnes"
    },
    {
      "affiliations": [
        "Division of Clinical Medicine, University of Sheffield, Sheffield, United Kingdom",
        "Department of Endocrinology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, United Kingdom"
      ],
      "name": "Miguel DeBono"
    },
    {
      "affiliations": [
        "Department of Internal Medicine and Therapeutics, University of Pavia, Pavia, Italy",
        "Istituti Clinici Scientifici Maugeri IRCCS, Gastroenterology Unit of Pavia Institute, Pavia, Italy"
      ],
      "name": "Federico Biagi"
    },
    {
      "affiliations": [
        "Department of Gastroenterology, AP-HP. Centre- Université Paris Cité, Hôpital Cochin, Paris, France",
        "Université de Paris, INSERM UMR 1163 and Imagine Institute, Laboratory of Intestinal Immunity, Université Paris Cité, Paris, France"
      ],
      "name": "Georgia Malamut"
    },
    {
      "affiliations": [
        "Department of Gastroenterology and Endoscopy, CELAC Network, AP-HP Centre, Hôpital Européen Georges Pompidou, Université de Paris, Cité and Institut National du Cancer, Paris, France"
      ],
      "name": "Christophe Cellier"
    },
    {
      "affiliations": [
        "Department of Surgery, Oncology, Gastroenterology, University of Padua, Padua, Italy"
      ],
      "name": "Fabiana Zingone"
    },
    {
      "affiliations": [
        "Department of Gastroenterology, Amsterdam University Medical Centre, Amsterdam, The Netherlands"
      ],
      "name": "Gerd Bouma"
    },
    {
      "affiliations": [
        "Center for Prevention and Diagnosis of Celiac Disease, Fondazione IRCCS, Granda Ospedale Maggiore Policlinico, Milan, Italy"
      ],
      "name": "Luca Elli"
    },
    {
      "affiliations": [
        "Medizinische Klinik Für Gastroenterologie, Charité—Universitätsmedizi, Berlin, Germany"
      ],
      "name": "Federica Branchi"
    },
    {
      "affiliations": [
        "Academic Unit of Gastroenterology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, United Kingdom",
        "Division of Clinical Medicine, University of Sheffield, Sheffield, United Kingdom"
      ],
      "name": "David Sanders"
    }
  ],
  "title": "O38 Coeliac disease outcomes of budesonide response in adults: COBRA STUDY from the European society for the study of coeliac disease (ESsCD)",
  "uid": "09e06df2-f293-5d90-b917-1d68ccda6bdd"
}