Background: Inflammatory bowel disease–associated liver diseases (IBD-LDs) include autoimmune hepatitis (AIH), primary sclerosing cholangitis (PSC), and an overlap syndrome. Prospective unbiased multicenter data regarding the frequency of IBD-LD in patients with pediatric inflammatory bowel disease (IBD) are lacking. We examined early alanine aminotransferase (ALT) and γ-glutamyl transpeptidase (GGT) elevations in children diagnosed as having IBD and assessed the likelihood of IBD-LD.
Methods: Data collected from the prospective observational Pediatric Inflammatory Bowel Disease Collaborative Research Group Registry enrolling children of age <16 years within 30 days of diagnosis. AIH, PSC, and overlap syndrome were diagnosed using local institutional criteria.
Results: A total of 1569 subjects had liver enzymes available. Of the total, 757 had both ALT and GGT, 800 had ALT only (no GGT), and 12 had GGT only (no ALT). Overall, 29 of 1569 patients (1.8%) had IBD-LD. IBD-LD was diagnosed in 1 of 661 (0.15%) of patients with both ALT and GGT ≤ 50 IU/L compared with 21 of 42 (50%) of patients with both ALT and GGT > 50 (odds ratio 660, P < 0.0001). Of the 29 patients with IBD-LD, 21 had PSC, 2 had AIH, and 6 had overlap syndrome. IBD-LD was more common in patients with ulcerative colitis and IBD-unclassified (indeterminate colitis) than in those with Crohn disease (4% vs 0.8%, respectively, P < 0.001).
Conclusions: Elevation of both ALT and GGT within 90 days after the diagnosis of IBD is associated with a markedly increased likelihood of IBD-LD. Both ALT and GGT levels should be measured in all of the pediatric patients newly diagnosed as having IBD.
*Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA
†Connecticut Children's Medical Center, Hartford
‡Hasbro Children's Hospital, Providence, RI
§I.W.K. Health Center, Halifax, Nova Scotia, Canada
||Hospital for Sick Children, Toronto, Ontario, Canada
¶Goryeb Children's Hospital, Morristown, NJ
#Children's Hospital of Wisconsin, Milwaukee
**Johns Hopkins Hospital, Baltimore, MD
††Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada
‡‡Children's Medical Center, Dayton, OH
§§Riley Hospital for Children, Indianapolis, IN
||||Nationwide Children's Hospital, Columbus, OH
¶¶Children's Hospital of Philadelphia, Philadelphia, PA
##Russell Children's Hospital, Birmingham, AB
***University of Minnesota, Minneapolis
†††University of North Carolina, Chapel Hill
‡‡‡Cincinnati Children's Medical Center, Cincinnati, OH
§§§Mayo Clinic, Rochester, MN
||||||Penn State Hershey Children's Hospital, Hershey, PA
¶¶¶Cohen Children's Medical Center, New Hyde Park, NY.
Address correspondence and reprint requests to Alka Goyal, Children's Hospital of Pittsburgh of UPMC, 4401 Penn Ave, Pittsburgh, PA 15224 (e-mail: firstname.lastname@example.org).
Received 31 October, 2013
Accepted 17 April, 2014
This article was partially supported by a grant from Janssen Ortho-Biotech.
The authors report no conflicts of interest.