Efficacy and Safety of Immunosuppressive Therapy for PBC–AIH Overlap Syndrome Accompanied by Decompensated Cirrhosis: A Real-World Study

Abstract EN

Aim.

To explore the efficacy and safety of immunosuppressive therapy for the treatment of primary biliary cirrhosis-autoimmune hepatitis (PBC-AIH) overlap syndrome accompanied by decompensated cirrhosis.

Methods.

A cohort study was performed to evaluate the usefulness of immunosuppressive therapy in this unique group.

This cohort study was performed between October 2013 and June 2017 and included 28 biopsy-proven patients diagnosed according to the Paris criteria.

The therapies included ursodeoxycholic acid (UDCA) alone (N=14) or in combination with immunosuppression (IS) therapy (N=14).

The primary endpoints were biochemical remission, liver-related adverse events, transplant-free survival, and drug side-effects.

Results.

The frequency of biochemical remission for the AIH features was significantly higher in the UDCA+IS group than in the UDCA-only group (60.0 versus 9.1%, P=0.024) after 12 months of therapy but not after 3 and 6 months (28.6 versus 0%, P=0.165; 35.7 versus 7.1%, P=0.098).

The rates of liver-related adverse events were lower in the combined group (2/14 versus 9/14, P=0.018).

The Kaplan-Meier estimate showed that the transplant-free survival was distinct between the two groups (P=0.019).

In the UDCA+IS group, mild and transient leukopenia occurred in two patients receiving azathioprine (AZA), and an infection was observed in one patient receiving mycophenolate mofetil (MMF).

Conclusions.

PBC-AIH patients with decompensated cirrhosis receiving a combination of UDCA and immunosuppressors presented with higher biochemical remission rates and experienced fewer liver-related adverse events, implying that the combined treatment might be a better therapeutic option for strictly defined decompensated PBC-AIH overlap syndrome.