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

Benedetta Terziroli Beretta-Piccoli; Luca Mazzucchelli; Chiara Taiana; Giuseppe Mossi; Corrado Usai; Diego Vergani; Giorgina Mieli-Vergani

doi:10.1016/j.jtauto.2019.100033

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

J Transl Autoimmun. 2019 Dec 17:3:100033. doi: 10.1016/j.jtauto.2019.100033. eCollection 2020.

Authors

Benedetta Terziroli Beretta-Piccoli^{1

2}, Luca Mazzucchelli³, Chiara Taiana⁴, Giuseppe Mossi⁵, Corrado Usai⁶, Diego Vergani², Giorgina Mieli-Vergani⁷

Affiliations

¹ Epatocentro Ticino, Lugano, Switzerland.
² Institute of Liver Studies, MowatLabs, King's College Hospital, London, UK.
³ Cantonal Pathology Institute, Locarno, Switzerland.
⁴ Studio Medico, Canobbio, Switzerland.
⁵ Studio Medico, Maggia, Switzerland.
⁶ Radiology Department, Regional Hospital EOC, Locarno, Switzerland.
⁷ Paediatric Liver, GI and Nutrition Centre, MowatLabs, King's College Hospital, London, UK.

Abstract

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.

Keywords: Autoimmune hepatitis; Normal serum alkaline phosphatase level; Primary biliary cholangitis; Primary sclerosing cholangitis; Variant syndrome.

Publication types

Case Reports