Open AccessUnique Inflammatory Bowel Disease Phenotype of Pediatric Primary Sclerosing Cholangitis
Author(s) -
Shiau Henry,
Ihekweazu Faith D.,
Amin Mansi,
Fofanova Tatiana,
Miloh Tamir,
Kellermayer Richard
Publication year - 2017
Publication title -
journal of pediatric gastroenterology and nutrition
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.206
H-Index - 131
eISSN - 1536-4801
pISSN - 0277-2116
DOI - 10.1097/mpg.0000000000001531
Subject(s) - medicine , primary sclerosing cholangitis , pancolitis , inflammatory bowel disease , gastroenterology , ulcerative colitis , calprotectin , faecal calprotectin , colectomy , disease , colonoscopy , colorectal cancer , cancer
ABSTRACT 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.