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      Development of the Crohn's disease digestive damage score, the Lémann score

      review-article
      , MD 1 , , MD 2 , , MD, PhD 3 , , MD 4 , , MD, PhD 5 , , MD, BS 6 , , MD 7 , , MD, PhD 8 , , MD 9 , , MD, PhD 10 , , MD, PhD 11 , , MD, MSc 12 , , MD 13 , , MD 14 , , MD, PhD 15 , , MD 16 , , MD, DMSci 17 , , MD, PhD 18 , , MD, PhD 19 , , MD, PhD 20 , , MD 21 , , MD 22 , , MD 23 , , MD 24 , , MD 25 , , DPhil, FRCP 26 , , MD, PhD 27 , , MD 28 , , PhD 29 , , MD, PhD 30 , , MD, PhD 1
      Inflammatory Bowel Diseases
      Wiley Subscription Services, Inc., A Wiley Company
      Crohn's disease, illness index severity, magnetic resonance imaging

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          Abstract

          Crohn's disease (CD) is a chronic progressive destructive disease. Currently available instruments measure disease activity at a specific point in time. An instrument to measure cumulative structural damage to the bowel, which may predict long-term disability, is needed. The aim of this article is to outline the methods to develop an instrument that can measure cumulative bowel damage. The project is being conducted by the International Program to develop New Indexes in Crohn's disease (IPNIC) group. This instrument, called the Crohn's Disease Digestive Damage Score (the Lémann score), should take into account damage location, severity, extent, progression, and reversibility, as measured by diagnostic imaging modalities and the history of surgical resection. It should not be “diagnostic modality driven”: for each lesion and location, a modality appropriate for the anatomic site (for example: computed tomography or magnetic resonance imaging enterography, and colonoscopy) will be used. A total of 24 centers from 15 countries will be involved in a cross-sectional study, which will include up to 240 patients with stratification according to disease location and duration. At least 120 additional patients will be included in the study to validate the score. The Lémann score is expected to be able to portray a patient's disease course on a double-axis graph, with time as the x-axis, bowel damage severity as the y-axis, and the slope of the line connecting data points as a measure of disease progression. This instrument could be used to assess the effect of various medical therapies on the progression of bowel damage. (Inflamm Bowel Dis 2011)

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          Most cited references40

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          Predictability of the postoperative course of Crohn's disease.

          Eighty-nine patients who had been treated by ileal resection for Crohn's disease between 1979 and 1984 were included in a prospective cohort follow up to study the natural course of early postoperative lesions. Recurrent lesions were observed endoscopically in the neoterminal ileum within 1 year of surgery in 73% of the patients, although only 20% of the patients had symptoms. Three years after surgery, the endoscopic recurrence rate had increased to 85% and symptomatic recurrence occurred in 34%. The ultimate course of the disease was best predicted by the severity of the early postoperative lesions, as observed at ileoscopy. Clinical parameters that influenced outcome were preoperative disease activity, the indication for surgery, and the number of surgical resections. When patients were stratified for preoperative disease activity, the severity of lesions found at endoscopy remained a strong predictive factor for symptomatic recurrence. In 22 other patients submitted to "curative" ileal resection and ileocolonic anastomosis, the segment to be used as neoterminal ileum was carefully examined during surgery, and two large biopsies were taken before making the anastomosis. An ileoscopy was performed 6 months after surgery. Although all patients had a macroscopically normal neoterminal ileum and 19 had entirely normal biopsies at the time of surgery, 21 patients were found at ileoscopy to have developed ileitis involving a 15-cm segment (range, 4-30 cm), and 20 had unequivocal microscopic lesions on biopsies. These studies suggest that early lesions in the neoterminal ileum after Crohn's resection do not originate from microscopic inflammation present in this bowel segment at the time of surgery. The early postoperative lesions in the neoterminal ileum seem to be a suitable model to study the pathogenesis of Crohn's disease and also to evaluate new therapeutic modalities, either to prevent development of these early lesions or to treat progressive recurrence.
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            The natural history of adult Crohn's disease in population-based cohorts.

            Natural history studies provide invaluable data on the disease course. First, they help define the end points for clinical trials that are designed to test drugs for the end point of disease modification in chronic disabling diseases. Natural history studies can also help to identify subsets of patients in whom the disease prognosis can be stratified according to clinical features. This comprehensive review summarizes our current knowledge of the natural history of Crohn's disease in adults as reported in population-based studies that include long-term follow-up results. We conducted a literature search of English and non-English language publications listed in the electronic databases of MEDLINE (source PUBMED, 1935 to December 2008). One-third of the patients had ileitis, colitis, or ileocolitis at the time of diagnosis. Disease location remained broadly stable over time. Up to one-third of the patients had evidence of a stricturing or penetrating intestinal complication at diagnosis, and half of all patients had experienced an intestinal complication within 20 years after diagnosis. Ten percent of the patients had prolonged clinical remission. Steroid dependency occurred in one-third of the patients, and surgery was required in one-third after initiation of steroid therapy. The annual incidence of hospitalizations was 20%. Half of the patients required surgery within 10 years after diagnosis. The risk of postoperative recurrence was 44-55% after 10 years. Crohn's disease is a disabling condition over time. The impact of changing treatment paradigms with increased use of immunosuppressants and biological agents on its natural history is poorly known.
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              Risk factors associated with progression to intestinal complications of Crohn's disease in a population-based cohort.

              We sought to assess the evolution of Crohn's disease behavior in an American population-based cohort. Medical records of all Olmsted County, Minnesota residents who were diagnosed with Crohn's disease from 1970 to 2004 were evaluated for their initial clinical phenotype, based on the Montreal Classification. The cumulative probabilities of developing structuring and/or penetrating complications were estimated using the Kaplan-Meier method. Proportional hazards regression was used to assess associations between baseline risk factors and changes in behavior. Among 306 patients, 56.2% were diagnosed between the ages of 17 and 40 years. Disease extent was ileal in 45.1%, colonic in 32.0%, and ileocolonic in 18.6%. At baseline, 81.4% had nonstricturing nonpenetrating disease, 4.6% had stricturing disease, and 14.0% had penetrating disease. The cumulative risk of developing either complication was 18.6% at 90 days, 22.0% at 1 year, 33.7% at 5 years, and 50.8% at 20 years after diagnosis. Among 249 patients with nonstricturing, nonpenetrating disease at baseline, 66 changed their behavior after the first 90 days from diagnosis. Relative to colonic extent, ileal, ileocolonic, and upper GI extent were significantly associated with changes in behavior, whereas the association with perianal disease was barely significant. In a population-based cohort study, 18.6% of patients with Crohn's disease experienced penetrating or stricturing complications within 90 days after diagnosis; 50% experienced intestinal complications 20 years after diagnosis. Factors associated with development of complications were the presence of ileal involvement and perianal disease. Copyright © 2010 AGA Institute. Published by Elsevier Inc. All rights reserved.
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                Author and article information

                Journal
                Inflamm Bowel Dis
                ibd
                Inflammatory Bowel Diseases
                Wiley Subscription Services, Inc., A Wiley Company
                1078-0998
                1536-4844
                June 2011
                28 November 2010
                : 17
                : 6
                : 1415-1422
                Affiliations
                [1 ]simpleDepartment of Hepatogastroenterology, Hôpital Saint-Louis Paris, France
                [2 ]simpleDepartment of Gastroenterology and Nutrition, Hôpital Saint-Antoine Paris, France
                [3 ]simpleDepartment of Gastroenterology, Instituto Clinico Humanitas Rozzano, Milan, Italy
                [4 ]simpleDivision of Gastroenterology, University of California San Diego La Jolla, California
                [5 ]simpleDepartment of Radiology, Hôpital Saint-Antoine Paris, France
                [6 ]simpleDepartment of Radiology, Mayo Clinic Rochester, Minnesota, USA
                [7 ]simpleDepartment of Gastroenterology, Rambam Health Care Campus Bat Galim, Haifa, Israel
                [8 ]simpleImelda GI Clinical Research Center Bonheiden, Belgium
                [9 ]simpleRobarts Research Institute, University of Western Ontario London, Ontario, Canada
                [10 ]simpleDivision of Gastroenterology and Hepatology, Department of Internal Medicine, Keio University School of Medicine Tokyo, Japan
                [11 ]simpleDepartment of Gastroenterology and Hepatology, Leiden University Medical Center Leiden, The Netherlands
                [12 ]simpleUniversity of Toronto and Division of Gastroenterology, St. Michael's Hospital Toronto, Ontario, Canada
                [13 ]simpleStVincent's Hospital & University of Melbourne Melbourne, Australia
                [14 ]simpleDivision of Gastroenterology and Hepatology, Mayo Clinic Rochester, Minnesota, USA
                [15 ]simpleDepartment of Hepatogastroenterology, Centre Hospitalier Universitaire de Liège, Liège University Liège, Belgium
                [16 ]simpleDepartment of Gastroenterology and Hepatology, Centre Hospitalier Universitaire Vaudois and University of Lausanne Lausanne, Switzerland
                [17 ]simpleDepartment of Medical Gastroenterology C, Herlev Hospital, University of Copenhagen Denmark
                [18 ]simpleAkershus University Hospital, Dept of GI Surgery, University in Oslo Norway
                [19 ]simpleGastroenterology Eepartment, Hospital Clinic of Barcelona Barcelona, Spain
                [20 ]simpleDepartment of Hepatogastroenterology, Centre Hospitalier Universitaire de Nancy Vandoeuvre-Lès-Nancy, France
                [21 ]simpleUniv Klinik Innere Medizin III Vienna, Austria
                [22 ]simpleMGH Crohn's and Colitis Center and Gastrointestinal Unit, Massachusetts General Hospital, Harvard Medical School Boston, Massachusetts, USA
                [23 ]simpleCEA Hospital Board, University - Hospital Frankfurt/Main Frankfurt, Germany
                [24 ]simpleInstitute of Clinical Molecular Biology, Christian-Albrechts University Kiel, Germany
                [25 ]simpleChristian Doppler Research Laboratory for Gut Inflammation, Medical University Innsbruck Austria
                [26 ]simpleTranslational Gastroenterology Unit, John Radcliffe Hospital Oxford, UK
                [27 ]simpleDivision of Gastroenterology, University of Leuven Hospitals Leuven, Belgium
                [28 ]simpleGastroenterology and Gastrointestinal Endoscopy Unit, IRCCS Policlinico San Donato & University of Milan San Donato Milanese, Italy
                [29 ]simpleINSERM U717, Biostatistics and Clinical Epidemiology, Hôpital Saint-Louis Paris, France
                [30 ]simpleDepartment of Hepatogastroenterology, Hôpital Huriez, Centre Hospitalier Universitaire Lille, France
                Author notes

                Supported by an educational grant from Abbott Laboratories.

                Conflicts of interest: William Sandborn, Yehuda Chowers, Edward V. Loftus, Jr., Edouard Louis, Tom Oresland, Julián Panés, Walter Reinisch, Simon Travis, Maurizio Vecchi, Jean Frederic Colombel, and Marc Lemann, have received research support from and served as consultants to Abbott Laboratories.

                Re-use of this article is permitted in accordance with the Terms and Conditions set out at http://wileyonlinelibrary.com/onlineopen#OnlineOpen_Terms

                Reprints: Jacques Cosnes, MD, Service Hépato-Gastroentérologie et Nutrition, Hôpital Saint Antoine, 184 rue du Faubourg Saint-Antoine, 75012 Paris, France (e-mail: jacques.cosnes@ 123456sat.aphp.fr )

                Article
                10.1002/ibd.21506
                3116198
                21560202
                e2c3ac39-5186-4f03-a9f2-b23f704b5fa2
                Copyright © 2011 Crohn's & Colitis Foundation of America, Inc.

                Re-use of this article is permitted in accordance with the Creative Commons Deed, Attribution 2.5, which does not permit commercial exploitation.

                History
                Categories
                Clincal Review

                Gastroenterology & Hepatology
                illness index severity,crohn's disease,magnetic resonance imaging

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