title: Unique Inflammatory Bowel Disease Phenotype of Pediatric Primary Sclerosing Cholangitis: A Single-Center Study.
abstract: 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.
  
  
  
  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.
  
  
  
  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).
  
  
  
  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.
authors.affiliation: *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.
authors.forename: Henry
authors.initials: H
authors.lastname: Shiau
authors.forename: Faith D
authors.initials: FD
authors.lastname: Ihekweazu
authors.forename: Mansi
authors.initials: M
authors.lastname: Amin
authors.forename: Tatiana
authors.initials: T
authors.lastname: Fofanova
authors.forename: Tamir
authors.initials: T
authors.lastname: Miloh
authors.forename: Richard
authors.initials: R
authors.lastname: Kellermayer
mesh_terms: D015415:Biomarkers / Q000378:metabolism; D002648:Child; D015209:Cholangitis, Sclerosing / Q000150:complications / Q000175:diagnosis* / Q000378:metabolism / Q000628:therapy; D003093:Colitis, Ulcerative / Q000150:complications / Q000175:diagnosis* / Q000378:metabolism / Q000628:therapy; D003424:Crohn Disease / Q000150:complications / Q000175:diagnosis* / Q000378:metabolism / Q000628:therapy; D018450:Disease Progression; D005260:Female; D006801:Humans; D008297:Male; D010641:Phenotype; D011379:Prognosis; D012189:Retrospective Studies
chemical_list: D015415:Biomarkers