Back
A patient with primary biliary cholangitis, autoimmune hepatitis, and primary sclerosing cholangitis variant syndrome.
Journal article

A patient with primary biliary cholangitis, autoimmune hepatitis, and primary sclerosing cholangitis variant syndrome.

  • Terziroli Beretta-Piccoli B Epatocentro Ticino, Lugano, Switzerland.
  • Mazzucchelli L Cantonal Pathology Institute, Locarno, Switzerland.
  • Taiana C Studio Medico, Canobbio, Switzerland.
  • Mossi G Studio Medico, Maggia, Switzerland.
  • Usai C Radiology Department, Regional Hospital EOC, Locarno, Switzerland.
  • Vergani D Institute of Liver Studies, MowatLabs, King's College Hospital, London, UK.
  • Mieli-Vergani G Paediatric Liver, GI and Nutrition Centre, MowatLabs, King's College Hospital, London, UK.
Show more…
  • 2020-08-04
Published in:
  • Journal of translational autoimmunity. - 2020
Autoimmune hepatitis
Normal serum alkaline phosphatase level
Primary biliary cholangitis
Primary sclerosing cholangitis
Variant syndrome
English Overlap between autoimmune hepatitis (AIH) and either primary biliary cholangitis (PBC) or primary sclerosing cholangitis (PSC) is not rare and has extensively been reported. We herein report the first well documented case of triple overlap. A 68-year-old male patient presented with asymptomatic PBC including normal alkaline phosphatase serum level, developed AIH five years later, associated with magnetic resonance cholangiopancreatography biliary changes typical for PSC. Despite treatment with ursodeoxycholic acid and mycophenolate mofetil, owing to prednisone and azathioprine intolerance, he continued to have interface hepatitis and developed increasing fibrosis at follow-up liver biopsy. Our case report raises awareness for this rare and difficult to diagnose and treat clinical phenotype.
Language
  • English
Open access status
gold
Identifiers
Persistent URL
https://folia.unifr.ch/global/documents/13734
Statistics
Document views: 16 File downloads: