Prediction of outcome in autoimmune hepatitis and variant syndromes

Abstract

In general the prognosis of treated autoimmune hepatitis is comparable to the general population. The knowledge on risk factors predicting long term survival is still limited. Better knowledge on risk factors can lead to tailored treatment of patients in the future, preventing side effects in patients currently overtreated and preventing disease progression in patients currently undertreated.

At diagnosis age, ethnicity, cirrhosis and ALT level were the main predictors for long-term survival. During treatment ALT level but not IgG level was associated with long-term survival. In patients presenting with acute or acute severe AIH improvement of liver function within 2 weeks after start of treatment was the main predictor for survival. In patients with AIH-PBC variant syndrome more patients than the guidelines indicate are treated with a combination of immunosupression and ursodeoxycholic acid.

New treatment options are urgently needed in AIH which requires a better understanding of changes in the immune system. To our suprise complement did not play a role in autoimmune hepatitis. Using B-cell related cytokines, patients could be divided into immunological subgroups with each a different presentation, treatment response and outcome.