Primary biliary cholangitis : therapeutic and evolving aspects about 46 cases.

Background: Primary biliary cholangitis (PBC) is a rare autoimmune chronic liver disease whose prevalence is increasing. Medical treatment is based on ursodeoxycholic acid. It association with autoimmune hepatitis defined the overlap syndrome (OS).

Aim: It was to determine therapeutic and evolving characteristics of PBC and to compare them to those in subjects having an overlap syndrome.

Methods: This was a retrospective study of all the patients' files with PBC from hepato-gastroenterology department at Monastir hospital from April 1999 to November 2013.

Results: Forty six patients were included in our study: 43 women and 3 men with an average age of 55 years. OS was retained in 13 patients. Cirrhosis was retained in 21 patients. Thirty-eight patients were treated with ursodeoxycholic acid (UDCA) associated with corticosteroids and immunosuppressors in the case of OS. After an average follow-up of 50 months [13-169 months] the overall response rate to UDCA was 55%. This rate was comparable between the 2 groups isolated PBC and OS. It was lower in the cirrhosis group compared with non-cirrhotic patients (40% vs 65%, p=0,06). One patient underwent a liver transplantation. Three patients were died as a result of decompensated cirrhosis.

Conclusion: Our study highlights the frequency of cirrhosis at the time of PBC diagnosis, which may explain the low rate of response to UDCA. There is no difference in the therapeutic and evolving aspects between patients with isolated PBC and those with SC.