22/10/2025
Management of AIH with primary biliary cholangitis (overlap syndrome)
Patients with histological features of AIH but serological findings of primary biliary cirrhosis (i.e., antimitochondrial antibodies [AMA] directed towards enzymes in the 2-oxoacid dehydrogenase family) can rapidly progress to cirrhosis, so even asymptomatic patients should be treated.
A combination of a corticosteroid plus an immunosuppressant is usually preferred.
Patients with contraindications to immunosuppressant therapy (e.g., cytopenia, active malignancies, or TPMT deficiency) are treated with corticosteroid monotherapy.
Ursodeoxycholic acid should be given in combination with immunosuppressive therapy in patients with overlap syndrome.
