Biliary disease progression in childhood onset autoimmune liver disease: A 30-year follow-up into adulthood.

ABSTRACT:

Background & aims

Long-term follow-up studies of paediatric onset autoimmune liver disease (AILD) are invaluable in helping better understand the clinical course of disease. In day-to-day practice clinicians struggle with disease definitions whilst patients and parents lack clear prognostic information.

Methods

The clinical progression of 159 patients with childhood onset AILD between June 1990 and December 2013 was reviewed, capturing data up to adulthood (ending May 2021).

Results

Presentation with autoimmune hepatitis (AIH) was dominant (n = 119); biliary presentations accounted for 25%. During follow up, biliary disease progression confirmed by cholangiography and/or liver histology was observed frequently: 19.8% (20/101) patients with childhood onset AIH type 1 (

SUBMITTER: Warner S 

PROVIDER: S-EPMC10790098 | biostudies-literature | 2024 Feb

REPOSITORIES: biostudies-literature