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      Extraintestinal Manifestations of Inflammatory Bowel Disease

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          Abstract

          Article first published online 2 July 2015

          Abstract:

          Extraintestinal manifestations (EIM) in inflammatory bowel disease (IBD) are frequent and may occur before or after IBD diagnosis. EIM may impact the quality of life for patients with IBD significantly requiring specific treatment depending on the affected organ(s). They most frequently affect joints, skin, or eyes, but can also less frequently involve other organs such as liver, lungs, or pancreas. Certain EIM, such as peripheral arthritis, oral aphthous ulcers, episcleritis, or erythema nodosum, are frequently associated with active intestinal inflammation and usually improve by treatment of the intestinal activity. Other EIM, such as uveitis or ankylosing spondylitis, usually occur independent of intestinal inflammatory activity. For other not so rare EIM, such as pyoderma gangrenosum and primary sclerosing cholangitis, the association with the activity of the underlying IBD is unclear. Successful therapy of EIM is essential for improving quality of life of patients with IBD. Besides other options, tumor necrosis factor antibody therapy is an important therapy for EIM in patients with IBD.

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          The prevalence of extraintestinal diseases in inflammatory bowel disease: a population-based study.

          K. Yu, J.-B. Blanchard, P Rawsthorne … (2001)
          The aim of this study was to determine the prevalence of the major extraintestinal manifestations of inflammatory bowel disease (IBD) and their relation to disease diagnosis and gender. We used the population-based University of Manitoba IBD Database, which includes longitudinal files on all subjects of all health system contacts identified by International Classification of Diseases, 9th Revision, Clinical Modification codes for visit diagnosis. We extracted a cohort from our database, which included subjects with a known diagnosis of IBD for at least 10 yr. We then determined how many contacts each subject had for each of the following extraintestinal IBD-associated immune diseases: primary sclerosing cholangitis, ankylosing spondylitis, iritis/uveitis, pyoderma gangrenosum, and erythema nodosum. We calculated the prevalence of the extraintestinal diseases using an administrative definition of having at least five health system contacts for the diagnosis in question. This administrative definition has previously been validated in Crohn's disease and ulcerative colitis (UC). A total of 6.2% of patients with IBD had one of six major extraintestinal diseases studied in this report. Only 0.3% of patients had multiple extraintestinal diseases. Iritis/uveitis was the most common extraintestinal disease of all assessed (2.2% of women and 1.1% of men). Iritis/uveitis was more common among women, particularly those with UC (3.8%). Primary sclerosing cholangitis was most common among men with UC (3%). Ankylosing spondylitis was more common among men, and the highest rate was seen among men with Crohn's disease (2.7%). Pyoderma gangrenosum was more common in Crohn's (1.2%) with no gender predilection. Erythema nodosum was similarly present in Crohn's and UC but was more common among women (1.9%). The associations of immune mediated diseases in extraintestinal sites may help us to further our understanding of IBD pathogenesis, and it may help us in developing a paradigm of disease subsets.
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            Frequency and risk factors for extraintestinal manifestations in the Swiss inflammatory bowel disease cohort.

            Valérie Pittet, Pierluigi Ballabeni, Gerhard Rogler … (2010)
            Data on the frequency of extraintestinal manifestations (EIMs) in Crohn's disease (CD) and ulcerative colitis (UC) and analyses of their risk factors are scarce. We evaluated their prevalence and risk factors in a large nationwide cohort of inflammatory bowel disease (IBD) patients. IBD patients from an adult clinical cohort in Switzerland (Swiss IBD cohort study) were prospectively included. Data from validated physician enrolment questionnaires were analyzed. A total of 950 patients were included, 580 (61%) with CD (mean age 41 years) and 370 (39%) with UC (mean age 42 years). Of these, 249 (43%) of CD and 113 (31%) of UC patients had one to five EIMs. The following EIMs were found: arthritis (CD 33%, UC 21%), aphthous stomatitis (CD 10%, UC 4%), uveitis (CD 6%, UC 4%), erythema nodosum (CD 6%, UC 3%), ankylosing spondylitis (CD 6%, UC 2%), psoriasis (CD 2%, UC 1%), pyoderma gangrenosum (CD and UC each 2%), and primary sclerosing cholangitis (CD 1%, UC 4%). Multiple logistic regression identified the following risk factors for ongoing EIM in CD: active disease (odds ratio (OR)=1.95, 95% confidence interval (CI)=1.17-3.23, P=0.01), and positive IBD family history (OR=1.77, 95% CI=1.07-2.92, P=0.025). No risk factors were identified in UC patients. EIMs are a frequent problem in CD and UC patients. Active disease and positive IBD family history are associated with ongoing EIM in CD patients. Identification of EIM prevalence and associated risk factors may result in increased awareness for this problem and thereby facilitating their diagnosis and therapeutic management.
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              PSC-IBD: a unique form of inflammatory bowel disease associated with primary sclerosing cholangitis.

              William J Sandborn, Alan R Zinsmeister, Edward V Loftus … (2004)
              Inflammatory bowel disease associated with primary sclerosing cholangitis (PSC-IBD) may have a high prevalence of rectal sparing, backwash ileitis, and colorectal neoplasia. To describe the clinical features and outcomes of PSC-IBD and compare these to a group of chronic ulcerative colitis (CUC) patients. The medical records of all patients with PSC-IBD evaluated at the Mayo Clinic Rochester between 1987 and 1992 were abstracted for information on endoscopic and histological features, colorectal neoplasia, surgery, and other clinical outcomes. Patients referred for colorectal neoplasia and those who did not undergo colonoscopy with biopsies were excluded. A control group of CUC patients matched for sex, duration of IBD at first clinic visit, and calendar year of first clinic visit was identified, and similar information was abstracted. Seventy one PSC-IBD patients and 142 CUC patients without PSC were identified. Rectal sparing and backwash ileitis were more common in the PSC-IBD group (52% and 51%, respectively) than in controls (6% and 7%, respectively). Overall, colorectal neoplasia developed in 18 cases and 15 controls, including 11 cancers (seven cases and four controls). An increased risk of colorectal neoplasia or death was not detected in a matched analysis. Although the cumulative incidence of colorectal neoplasia was higher in cases (33%) than in controls (13%) at five years, this was of borderline statistical significance (p=0.054, unmatched log rank test). Overall survival from first clinic visit was significantly worse among cases (79% v 97%) at five years (p<0.001, unmatched log rank test). PSC-IBD is frequently characterised by rectal sparing and backwash ileitis. Colorectal neoplasia develops in a substantial fraction and overall survival is worse. PSC-IBD may represent a distinct IBD phenotype.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Inflamm Bowel Dis
                Journal ID (iso-abbrev): Inflamm. Bowel Dis
                Journal ID (publisher-id): ibd
                Title: Inflammatory Bowel Diseases
                Publisher: Lippincott Williams & Wilkins
                ISSN (Print): 1078-0998
                ISSN (Electronic): 1536-4844
                Publication date (Electronic): 02 July 2015
                Publication date (Print): August 2015
                Volume: 21
                Issue: 8
                Pages: 1982-1992
                Affiliations
                [* ]Department of Medicine, Division of Gastroenterology and Hepatology, University Hospital Zurich, Zurich, Switzerland;
                [† ]Department of Medicine, Division of Gastroenterology and Hepatology, Triemlispital Zurich, Zurich, Switzerland;
                [‡ ]Department of Medicine, Division of Gastroenterology and Hepatology, Centre Hospitalier Universitaire Vaudois (CHUV), Lausanne, Switzerland;
                [§ ]1st Department of Medicine, Semmelweis University, Budapest, Hungary; and
                [‖ ]Department of Dermatology, University Hospital Zurich, Zurich, Switzerland.
                Author notes
                Reprints: Stephan R. Vavricka, MD, Department of Internal Medicine, Division of Gastroenterology, Triemli Hospital, Birmensdorferstrasse 497, CH-8063 Zurich, Switzerland (e-mail: stephan.vavricka@ 123456usz.ch ).
                Article
                Publisher ID: IBD-14-0882 Accession ID: 00031
                DOI: 10.1097/MIB.0000000000000392
                PMC ID: 4511685
                PubMed ID: 26154136
                SO-VID: d37982c6-ebeb-44b8-a5d7-e3ce4f36d851
                Copyright © Copyright © 2015 Crohn's & Colitis Foundation of America, Inc.
                License:

                This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 06 November 2014
                Date accepted : 09 February 2015
                Categories
                Subject: Clinical Review Articles
                Custom metadata
                OPEN-ACCESS TRUE

                ScienceOpen disciplines: Gastroenterology & Hepatology
                Keywords: extraintestinal manifestations,inflammatory bowel disease,arthritis,uveitis
                Data availability:
                ScienceOpen disciplines: Gastroenterology & Hepatology
                Keywords: extraintestinal manifestations, inflammatory bowel disease, arthritis, uveitis

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