Liver transplantation for overlap syndromes of autoimmune liver diseases

The term overlap syndrome describes variant forms of autoimmune hepatitis (AIH) that present in combination with either characteristics of primary biliary cirrhosis (PBC), or primary sclerosing cholangitis (PSC). This study analysed the outcomes and evidence of recurrent liver disease after liver transplantation in patients with overlap syndromes compared with patients transplanted for single autoimmune liver disease.

We evaluated 231 adult patients who received a liver transplant as a result of autoimmune liver diseases; including 103 with PBC, 84 with PSC, 32 with AIH and 12 with overlap syndrome (7 AIH-PBC and 5 AIH-PSC).

Patients with overlap syndromes had a higher probability of recurrence than patients with a single autoimmune liver disease (5 years: 53% vs. 17%; 10 years 69% vs. 29%, P = 0.001). Furthermore, median time for recurrence in overlap syndrome was shorter when compared with patients with single autoimmune liver disease (67 ± 20 vs. 172 ± 9 months, P = 0.001). The diagnosis of overlap syndrome was independently associated with a higher risk to develop recurrent disease than patients transplanted with a single disease (HR 3.39, P = 0.007). Median graft survival for overlap syndrome was 123 ± 16 months and 180 ± 8 months in patients with single autoimmune liver diseases (P = 0.9), and median patient survival for overlap syndrome was 135 ± 13 months and 193 ± 8 months in patients with single autoimmune liver disease (P = 0.6).

Patients that received an allograft for end-stage liver disease secondary to overlap syndrome had a higher rate of disease recurrence when compared with transplant recipients with single autoimmune liver disorders, but the overall survival was comparable.