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Extraintestinal Manifestations of Pediatric Inflammatory Bowel Disease and Their Relation to Disease Type and Severity

Dotson, Jennifer L; Hyams, Jeffrey S; Markowitz, James§; LeLeiko, Neal S||; Mack, David R; Evans, Jonathan S#; Pfefferkorn, Marian D**; Griffiths, Anne M††; Otley, Anthony R‡‡; Bousvaros, Athos§§; Kugathasan, Subra||||; Rosh, Joel R; Keljo, David##; Carvalho, Ryan S***; Tomer, Gitit†††; Mamula, Petar‡‡‡; Kay, Marsha H§§§; Kerzner, Benny||||||; Oliva-Hemker, Maria¶¶¶; Langton, Christine R; Crandall, Wallace

Journal of Pediatric Gastroenterology & Nutrition:
doi: 10.1097/MPG.0b013e3181ca4db4
Original Articles: Gastroenterology

Objectives: Although it is known that extraintestinal manifestations (EIMs) commonly occur in pediatric inflammatory bowel disease (IBD), little research has examined rates of EIMs and their relation to other disease-related factors in this population. The purpose of this study was to determine the rates of EIMs in pediatric IBD and examine correlations with age, sex, diagnosis, disease severity, and distribution.

Patients and Methods: Data were prospectively collected as part of the Pediatric IBD Collaborative Research Group Registry, an observational database enrolling newly diagnosed IBD patients <16 years old since 2002. Rates of EIM (occurring anytime during the period of enrollment) and the aforementioned variables (at baseline) were examined. Patients with indeterminate colitis were excluded from the analysis given the relatively small number of patients.

Results: One thousand nine patients were enrolled (mean age 11.6 ± 3.1 years, 57.5% boys, mean follow-up 26.2 ± 18.2 months). Two hundred eighty-five (28.2%) patients experienced 1 or more EIMs. Eighty-seven percent of EIM occurred within the first year. Increased disease severity at baseline (mild vs moderate/severe) was associated with the occurrence of any EIM (P < 0.001), arthralgia (P = 0.024), aphthous stomatitis (P = 0.001), and erythema nodosum (P = 0.009) for both Crohn disease (CD) and ulcerative colitis (UC) during the period of follow-up. Statistically significant differences in the rates of EIMs between CD and UC were seen for aphthous stomatitis, erythema nodosum, and sclerosing cholangitis.

Conclusions: EIMs as defined in this study occur in approximately one quarter of pediatric patients with IBD. Disease type and disease severity were commonly associated with the occurrence of EIMs.

Author Information

Nationwide Children's Hospital, Columbus, OH, USA

Connecticut Children's Medical Center, Hartford, CT, USA

§North Shore Long Island Jewish Health System, New Hyde Park, NY, USA

||Hasbro Children's Hospital, Providence, RI, USA

Children's Hospital of Eastern Ontario, Ottawa, Canada

#Nemours Clinic, Jacksonville, FL, USA

**Riley Hospital for Children, Indianapolis, IN, USA

††Hospital for Sick Children, Toronto, Ontario, Canada

‡‡IWK Health Centre, Halifax, Nova Scotia, Canada

§§Children's Hospital Boston, Boston, MA, USA

||||Children's HealthCare of Atlanta, Atlanta, GA, USA

¶¶Morristown Memorial Hospital, Morristown, NY, USA

##Children's Hospital, Pittsburgh, PA, USA

***Children's Medical Center, Dayton, OH, USA

†††Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA

‡‡‡Children's Hospital of Philadelphia, Philadelphia, PA, USA

§§§Cleveland Clinic, Cleveland, OH, USA

||||||Children's National Medical Center, Washington, DC, USA

¶¶¶Johns Hopkins Medical Institute, Baltimore, MD, USA.

Received 8 April, 2009

Accepted 12 October, 2009

Address correspondence and reprint requests to Jennifer L. Dotson, MD, Nationwide Children's Hospital, 700 Children's Dr, Columbus, OH 43205 (e-mail:

The authors report no conflicts of interest.

Copyright 2010 by ESPGHAN and NASPGHAN