Clinical Course and Outcome of Autoimmune Hepatitis/Primary Sclerosing Cholangitis Overlap Syndrome

TLDR

Autoimmune hepatitis/primary sclerosing cholangitis (AIH/PSC) overlap syndrome is a relatively uncommon variant of PSC. The study aimed to compare the natural history of AIH/PSC overlap syndrome with that of classical PSC. Forty‑one consecutive PSC patients were prospectively followed for at least two years, with AIH/PSC patients receiving immunosuppression plus ursodeoxycholic acid and classical PSC patients receiving ursodeoxycholic acid alone. Compared to classical PSC, AIH/PSC overlap patients were younger, had markedly higher AST, ALT, and IgG levels, experienced no deaths, had better survival, fewer liver transplants and neoplasms, and their Mayo score did not rise during follow‑up.

Abstract

Autoimmune hepatitis/primary sclerosing cholangitis (AIH/PSC) overlap syndrome is a relatively uncommon variant of PSC.To evaluate the natural history of AIH/PSC overlap syndrome compared to a group of "classical" PSC.Forty-one consecutive PSC patients, with a regular follow-up of at least 2 years, were prospectively included in the study. Among these, 7 fulfilled the criteria for AIH/PSC overlap syndrome.The AIH/PSC overlap group significantly differed from the "classical" PSC group in the following parameters: mean age at presentation (21.4 +/- 5.0 vs 32.3 +/- 10 years, p < 0.01), AST 191.0 +/- 14.8 vs 48.9 +/- 34.5 U/L, p < 0.005), ALT (357.0 +/- 26.5 vs 83.7 +/- 60.7 U/L, p < 0.005) and serum IgG (25.6 +/- 4.7 vs 12.9 +/- 6.0 mg/dl, p < 0.0001). The mean follow-up was similar in the 2 groups (93.3 +/- 65.9 vs 98.1 +/- 65.9 months respectively). Treatment included immunosuppression + ursodeoxycholic acid (UDCA) in the AIH/PSC overlap patients, and UDCA in the "classical" PSC group. Deaths were recorded only in the classical PSC group. The median survival in the latter group was 207 months (95% C.I. 87.6-326.4). The major events during the follow-up included: OLTx (1/7 vs 6/34), and neoplasms (only in the group of "classical" PSC). The new Mayo score prognostic index only increased significantly during follow-up in the "classical" PSC group (r2 0.8117, p < 0.01)Patients with AIH/PSC overlap syndrome seem to benefit from immunosuppression + UDCA therapy, survival is apparently better than in "classical" PSC condition.