Liver and Bile
Dig Liver Dis. 2023;55(11):1515–20
Long-term outcome of autoimmune hepatitis: Consecutive patient cohort and data on the second 20 years
Background: Mortality rates for autoimmune hepatitis (AIH) vary. Data are lacking beyond 20 years follow-up.
Aims: Analysis of a consecutively recruited large AIH cohort from a single non-transplant tertiary center in England and an overlapping cohort, already followed for ≥ 20 years.
Methods: The authors assessed 330 patients presenting 1987–2016 and 65 patients presenting 1971–1996 already followed for 20 years.
Results: Death/liver transplant rate was 51 ± 4% (all-cause) and 21 ± 4% (liver-related) over 20 years and was independently associated with: decompensation and lower serum alanine aminotransferase (ALT) at diagnosis; and failure of serum ALT normalization and higher relapse rate. There was excess mortality over the first year. Patients (n = 65) already followed for 20 years had similar subsequent rates of relapse, disease progression and mortality, to those followed from diagnosis. Azathioprine-intolerant patients (n = 23) switching to mycophenolate did not have higher mortality over 4 (1–17) years, than patients continuing azathioprine. Following immunosuppression withdrawal (n = 26), 6 patients (23%) relapsed with no liver-related deaths over 2.3 (0–23.1) years.
Conclusions: In this consecutive autoimmune hepatitis cohort, mortality was similar to that in national registry studies, disease progression continued after 20 years, and immunosuppression withdrawal did not compromise survival.