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: firstname.lastname@example.org
Received November 25, 2013
Accepted February 5, 2014