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Sequential presentation of primary biliary cirrhosis and autoimmune hepatitis

Efe, Cumalia; Ozaslan, Ersanb; Heurgué-Berlot, Alexandrac; Kav, Taylana; Masi, Chiarae; Purnak, Tugrulb; Torgutalp, Murata; Muratori, Luigie; Bresson-Hadni, Solanged; Thiéfin, Gérardc; Schiano, Thomas D.f; Muratori, Paoloe; Wahlin, Staffang

European Journal of Gastroenterology & Hepatology: May 2014 - Volume 26 - Issue 5 - p 532–537
doi: 10.1097/MEG.0000000000000075
Original Articles: Hepatitis

Background Primary biliary cirrhosis (PBC)-autoimmune hepatitis (AIH) overlap syndrome is used to describe the coexistence of both diseases, with either a sequential or a simultaneous presentation in the same patient. Available studies have focused on the simultaneous form, whereas there is limited information on sequential PBC-AIH. We carried out a retrospective study of patients who sequentially developed PBC-AIH overlap syndrome.

Methods The medical data of 1065 patients diagnosed with PBC (n=483) and AIH (n=582) were retrospectively analyzed.

Results A sequential development of PBC-AIH was observed in 19 (1.8%) patients after a mean of 6.5 (1–14) years of follow-up. AIH developed in 12 (2.5%) PBC patients, whereas PBC occurred in seven (1.2%) patients with AIH. The baseline serologic and histological findings of patients who developed PBC-AIH were similar to those of patients with typical PBC or AIH. Eighteen patients were treated with a combination of ursodeoxycholic acid (UDCA) and immunosuppression after the diagnosis of PBC-AIH was established. One patient showed a spontaneous resolution of hepatitic flare under UDCA therapy. Biochemical remission was achieved in 16 patients, whereas three progressed to decompensated cirrhosis and required liver transplantation.

Conclusion The sequential overlap of PBC-AIH can occur during the follow-up of patients with pure PBC or AIH. In our cohort, we could not identify any factors that predicted the development of this rare condition. The combination of UDCA and immunosuppression seems to be an appropriate therapy in the setting of PBC-AIH.

aDepartment of Gastroenterology, Hacettepe University

bDepartment of Gastroenterology, Numune Research and Education Hospital, Ankara, Turkey

cDepartment of Hepato-Gastroenterology, CHU Reims, Reims

dDepartment of Hepatology, Besançon, France

eDepartment of Clinical Medicine, Alma Mater Studiorum, University of Bologna, Bologna, Italy

fDivision of Liver Diseases, the Mount Sinai Medical Center, New York, New York, USA

gDepartment of Gastroenterology and Hepatology, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden

Correspondence to Cumali Efe, MD, Erzurum caddesi, Gül sokak 3/13, Cebeci 06100, Ankara, Turkey Tel: +90 505 502 5589; fax: +90 312 315 026; e-mail:

Received November 25, 2013

Accepted February 5, 2014

© 2014 Wolters Kluwer Health | Lippincott Williams & Wilkins