Impact of paediatric onset primary sclerosing cholangitis on clinical course and outcome of inflammatory bowel disease: a case-control population-based study in Finland

Abstract

Introduction and aim: The aim of this study was to investigate the outcome of a paediatric onset of inflammatory bowel disease (IBD) in a cohort of subjects with primary sclerosing cholangitis (PSC) and in a matched-age population-based control group without PSC.

Methods: We identified 28 IBD-PSC cases (median age at IBD diagnosis 12.5 years, 25–75th: 10–16 years) and selected three IBD controls for each case matched for age and year of IBD diagnosis. All data regarding the gastrointestinal tract and liver were collected at diagnosis and at last follow-up (median 15 years).

Results: At diagnosis the prevalence of pancolitis was similar between the groups (78% and 79%, respectively p = –.30), but histologic inflammation was milder in IBD-PSC (61% vs 30%, p = .06). At last follow-up (median age 29 years) pancolitis was less frequent (6% and 33%, respectively p = .04) and the remission higher (76% and 47%, respectively p = .08) in IBD-PSC patients than in IBD patients. Panproctolectomy (32% in IBD-PSC and 34% in IBD, p = 1.0) and the rate of pouchitis (62% and 70%, respectively p = .8) were similar.

Conclusions: The outcome of paediatric onset IBD in patients with PSC in adulthood seems to be comparable to those with IBD only.

Keywords:

• Primary sclerosing cholangitis
• autoimmune liver disease
• dysplasia
• colorectal cancer
• pouchitis
• ulcerative colitis

Acknowledgements

We thank RN Anne Nikkonen for helping in the collection of the data.

Disclosure statement

The authors have indicated they have no potential conflicts of interest to disclose.

Additional information

Funding

The study was supported by the Sigrid Jusélius Foundation, the Paediatric Research Foundation and the Helsinki University Hospital Research Fund.