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Unique Inflammatory Bowel Disease Phenotype of Pediatric Primary Sclerosing Cholangitis: A Single-Center Study

Shiau, Henry*,†; Ihekweazu, Faith D.; Amin, Mansi; Fofanova, Tatiana§; Miloh, Tamir; Kellermayer, Richard‡,||

Journal of Pediatric Gastroenterology and Nutrition: October 2017 - Volume 65 - Issue 4 - p 404–409
doi: 10.1097/MPG.0000000000001531
Original Articles: Gastroenterology: Inflammatory Bowel Disease

Objectives: In adults, primary sclerosing cholangitis (PSC), a cholestatic liver disease characterized by inflammation/fibrosis of intra/extrahepatic bile ducts, associates with a milder form of inflammatory bowel disease (IBD), particularly ulcerative colitis (UC). The pediatric PSC-IBD phenotype is less well characterized.

Methods: We performed a retrospective, single-center study examining patients with PSC-IBD at Texas Children's Hospital between 2000 and 2015. IBD-phenotype (Modified Montreal Classification), medications, laboratory values, endoscopic records, and IBD-based hospital admissions were collected. PSC-UC phenotype was compared to UC, non-PSC patients (n = 95) from Texas Children's Hospital. Elevated gamma-glutamyl transpeptidase levels were compared to calprotectin levels and IBD-flare activity, that is, gastrointestinal symptoms resulting in office/emergency department visits or hospital admission.

Results: Of 39 patients with PSC-IBD, 34 (87.2%) had UC (PSC-UC) and 5 (12.8%) had Crohn disease. Pancolitis was more common in PSC-UC than UC, non-PSC (96.3%, 64%, P = 0.0009). Patients with PSC-UC required less treatment with steroids (76.5%, 91.6%, P = 0.0326) or infliximab (8.8%, 37.9%, P = 0.0011), and fewer had at least 1 IBD-related hospital admission (32.4%, 63.2%, P = 0.0025) than UC, non-PSC. Progression to colectomy was significantly less (5.8%, 24.2%, P = 0.0223) in PSC-UC. Median diagnosis-to-colectomy time tended to be longer in PSC-UC (6.37, 2.5 years, P = 0.0792). In 2 smaller subsets, gamma-glutamyl transpeptidase did not correlate with calprotectin in PSC-UC (n = 11, P = 0.7922) and less strongly associated with IBD-flares in PSC-UC than UC, non-PSC (n = 33, n = 67; 15.2%, 41.8%, P = 0.0120).

Conclusions: Pediatric PSC appears to associate with milder pancolitic-UC. PSC and IBD activity do not appear to correlate. Our findings may provide useful information toward etiology and management of pediatric PSC-IBD.

*Department of Pediatrics, Baylor College of Medicine

Department of Pediatrics

Section of Pediatric Gastroenterology, Hepatology and Nutrition, Baylor College of Medicine, Texas Children's Hospital

§Translational Biology and Molecular Medicine, Center for Metagenomics and Microbiome Research, Baylor College of Medicine

||Children's Nutrition and Research Center, Houston, TX.

Address correspondence and reprint requests to Richard Kellermayer, MD, PhD, Section of Pediatric Gastroenterology, Hepatology and Nutrition, Baylor College of Medicine, 6621 Fannin St, CC1010.00 Houston, TX 77030-2399 (e-mail:

Received 21 July, 2016

Accepted 23 January, 2017

The authors report no conflicts of interest.

© 2017 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,