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Clinical features of type 1 and 2 refractory celiac disease: Results from a large cohort over a decade

  • Luca Elli
    Correspondence
    Corresponding author.
    Affiliations
    Department of Pathophysiology and Transplantation, University of Milan, Milan 20122, Italy

    Center for Prevention and Diagnosis of Celiac Disease and Division of Gastroenterology and Endoscopy, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via F. Sforza 35, Milan 20122, Italy
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  • Pietro Soru
    Affiliations
    Department of Pathophysiology and Transplantation, University of Milan, Milan 20122, Italy

    Center for Prevention and Diagnosis of Celiac Disease and Division of Gastroenterology and Endoscopy, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via F. Sforza 35, Milan 20122, Italy
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  • Leda Roncoroni
    Affiliations
    Department of Biomedical, Surgical and Dental Sciences, University of Milan, Milan 20133, Italy
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  • Francesca Gaia Rossi
    Affiliations
    Division of Hematology, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via F. Sforza 35, Milan 20122, Italy
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  • Valeria Ferla
    Affiliations
    Division of Hematology, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via F. Sforza 35, Milan 20122, Italy
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  • Luca Baldini
    Affiliations
    Division of Hematology, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via F. Sforza 35, Milan 20122, Italy

    Department of Oncology and Hemato-Oncology, University of Milan, Milan 20122, Italy
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  • Nicoletta Nandi
    Affiliations
    Department of Pathophysiology and Transplantation, University of Milan, Milan 20122, Italy

    Center for Prevention and Diagnosis of Celiac Disease and Division of Gastroenterology and Endoscopy, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via F. Sforza 35, Milan 20122, Italy
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  • Lucia Scaramella
    Affiliations
    Center for Prevention and Diagnosis of Celiac Disease and Division of Gastroenterology and Endoscopy, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via F. Sforza 35, Milan 20122, Italy
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  • Alice Scricciolo
    Affiliations
    Center for Prevention and Diagnosis of Celiac Disease and Division of Gastroenterology and Endoscopy, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via F. Sforza 35, Milan 20122, Italy
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  • Alessandro Rimondi
    Affiliations
    Department of Pathophysiology and Transplantation, University of Milan, Milan 20122, Italy

    Center for Prevention and Diagnosis of Celiac Disease and Division of Gastroenterology and Endoscopy, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via F. Sforza 35, Milan 20122, Italy
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  • Nicola Fusco
    Affiliations
    Biobank for Translational Medicine Unit (B4MED), Division of Pathology, IEO European Institute of Oncology, University of Milan, Via Ripamonti 435, 20132 Milan, Italy
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  • Giorgio Alberto Croci
    Affiliations
    Department of Pathophysiology and Transplantation, University of Milan, Milan 20122, Italy

    Division of Pathology, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via F. Sforza 35, Milan 20122, Italy
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  • Umberto Gianelli
    Affiliations
    Department of Pathophysiology and Transplantation, University of Milan, Milan 20122, Italy

    Division of Pathology, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via F. Sforza 35, Milan 20122, Italy
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  • Lilla Cro
    Affiliations
    Servizio di Citofluorimetria, Laboratorio Centrale, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
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  • Marzia Barbieri
    Affiliations
    Servizio di Citofluorimetria, Laboratorio Centrale, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
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  • Vincenza Lombardo
    Affiliations
    Center for Prevention and Diagnosis of Celiac Disease and Division of Gastroenterology and Endoscopy, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via F. Sforza 35, Milan 20122, Italy
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  • Andrea Costantino
    Affiliations
    Department of Pathophysiology and Transplantation, University of Milan, Milan 20122, Italy

    Center for Prevention and Diagnosis of Celiac Disease and Division of Gastroenterology and Endoscopy, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via F. Sforza 35, Milan 20122, Italy
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  • Valentina Vaira
    Affiliations
    Department of Pathophysiology and Transplantation, University of Milan, Milan 20122, Italy

    Division of Pathology, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via F. Sforza 35, Milan 20122, Italy
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  • Stefano Ferrero
    Affiliations
    Department of Biomedical, Surgical and Dental Sciences, University of Milan, Milan 20133, Italy

    Division of Pathology, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via F. Sforza 35, Milan 20122, Italy
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  • Gian Eugenio Tontini
    Affiliations
    Department of Pathophysiology and Transplantation, University of Milan, Milan 20122, Italy

    Center for Prevention and Diagnosis of Celiac Disease and Division of Gastroenterology and Endoscopy, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via F. Sforza 35, Milan 20122, Italy
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  • Giulio Barigelletti
    Affiliations
    Cancer Registry Unit, Fondazione IRCCS Istituto Nazionale dei Tumori Milano, Milan Italy
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  • Sabrina Fabiano
    Affiliations
    Cancer Registry Unit, Fondazione IRCCS Istituto Nazionale dei Tumori Milano, Milan Italy
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  • Luisa Doneda
    Affiliations
    Department of Biomedical, Surgical and Dental Sciences, University of Milan, Milan 20133, Italy
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  • Maurizio Vecchi
    Affiliations
    Department of Pathophysiology and Transplantation, University of Milan, Milan 20122, Italy

    Center for Prevention and Diagnosis of Celiac Disease and Division of Gastroenterology and Endoscopy, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via F. Sforza 35, Milan 20122, Italy
    Search for articles by this author
Published:September 09, 2022DOI:https://doi.org/10.1016/j.dld.2022.08.022

      Abstract

      Objectives

      Refractory celiac disease (RCeD) is a rare complication of celiac disease (CeD) with a severe prognosis. We describe a cohort of patients with RCeD, their clinical and histological features at diagnosis, after therapy and at lymphoma onset, and the rate and causes of death over a 17-year follow-up.

      Methods

      We retrospectively enrolled RCeD-I and RCeD-II patients attending our center between January 2002 and October 2019. Medical data were collected at diagnosis and during monitoring. Response to therapy, changes in RCeD molecular markers, number of hospitalizations, discharge diagnosis, and cause and date of death were evaluated. The control cohort consisted of 1015 responsive CeD patients.

      Results

      Compared with RCeD-I, RCeD-II more frequently exhibits diarrhea (83 vs 64%), anemia (61 vs 50%), hypoalbuminemia (70 vs 21%), parenteral nutrition need (48 vs 7%), ulcerative jejuno-ileitis (7 vs 39%), and extended small intestinal atrophy (62 vs 21%). One RCeD-I and six RCeD-II patients developed lymphoma. Ten RCeD-II patients died, four from lymphoma progression. Among RCeD-II patients, atrophy extension was the only parameter correlated with hypoalbuminemia and mortality.

      Conclusions

      Clinical severity, response to therapy, and mortality differ between RCeD-I and RCeD-II. Atrophy extension, evaluated at capsule endoscopy, was associated with disease severity and mortality.

      Keywords

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