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The characteristics of pediatric ulcerative colitis with primary sclerosing cholangitis: A single-center study in
Mu-Ming Chien1, Mei-Hwei Chang2, Kai-Chi Chang3
1Department of Pediatrics, National Taiwan University Children's Hospital, National Taiwan University, Taipei, Taiwan; Department of Pediatrics, Taipei Medical University Hospital, Taipei, Taiwan.
Insights
Pediatric patients with primary sclerosing cholangitis and ulcerative colitis (PSC-UC) had milder colitis. However, they required more immunomodulators and were less likely to achieve long-term steroid-free status for liver disease control.
Area of Science:
- Gastroenterology
- Hepatology
- Pediatric Medicine
Background:
- Primary sclerosing cholangitis (PSC) is a chronic liver disease often co-occurring with ulcerative colitis (UC).
- The clinical presentation of PSC in pediatric UC patients requires further investigation.
Purpose of the Study:
- To compare the clinical characteristics of pediatric UC patients with and without PSC.
- To analyze differences in disease severity, medication use, and long-term outcomes between PSC-UC and non-PSC UC groups.
Main Methods:
- Retrospective analysis of pediatric UC patients diagnosed between 2006 and 2017 at a Taiwanese tertiary center.
- Inclusion of patients with and without PSC, collecting data on clinical and endoscopic UC severity, medications, and laboratory parameters.
Main Results:
- Five pediatric patients with PSC-UC and 26 with UC alone were analyzed.
- PSC-UC patients exhibited significantly lower UC endoscopic severity (Mayo score and UC Endoscopic Index of Severity).
- PSC-UC patients had higher immunomodulator use (100% vs. 42.3%) and were less likely to be steroid-free (20% vs. 84.6%) at study end.
Conclusions:
- Pediatric PSC-UC is associated with less severe colitis compared to UC alone.
- Despite milder colitis, PSC-UC patients necessitate immunomodulator therapy and show reduced likelihood of long-term steroid-free status, likely for managing associated liver disease.
Background:
Primary sclerosing cholangitis (PSC) is often associated with ulcerative colitis (UC). We investigated the clinical characteristics of pediatric UC patients with and without PSC.
Methods:
We retrospectively recruited children with UC, with and without PSC, from 2006 to 2017 in a tertiary center in Taiwan. The clinical data of the patients, including clinical and endoscopic UC severity scores, medications, and laboratory parameters, were analyzed.
Results:
We recruited five children with PSC-UC (PSC-UC group), and 26 with UC alone (non-PSC UC group) in this retrospective analysis. Among the patients with PSC-UC, four (80%) were compatible with definite or probable autoimmune sclerosing cholangitis (ASC). The UC Endoscopic Index of Severity (5.00 vs. 9.00, P = 0.003) and Mayo score (4.00 vs. 8.00, P = 0.014) were significantly lower in the PSC-UC group than the non-PSC UC group. The prevalence of immunomodulator use was significantly higher in the PSC-UC than the non-PSC UC group (100% vs. 42.3%, P = 0.043), but there was no difference regarding steroids, mesalamine, or biologics. At the end of the study, significantly fewer patients were steroid-free in the PSC-UC than the non-PSC UC group (20.0% vs. 84.6%, P = 0.010).
Conclusions:
Pediatric patients with PSC-UC had less severe colitis than those with UC alone in terms of the clinical activity index and endoscopic severity index, but they were more likely to need an immunomodulator and less likely to be steroid-free in the long term, for the control of liver disease.
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