Treatment of Autoimmune Hepatitis with Overlap Syndrome — AIH-PBC Overlap or AIH-PSC Overlap
Clinical Scenario
A subset of patients with autoimmune hepatitis (AIH) concurrently meet diagnostic criteria for a cholestatic liver disease — either primary biliary cholangitis (PBC) or primary sclerosing cholangitis (PSC). These overlap syndromes (AIH-PBC overlap and AIH-PSC overlap) represent a distinct clinical situation requiring a treatment strategy that addresses both the inflammatory and cholestatic disease components.
Overlap with Primary Biliary Cholangitis or Primary Sclerosing Cholangitis
Patients presenting with features of both AIH and PBC — or AIH and PSC — carry a dual disease burden. The overlap phenotype is an established indication to expand the standard AIH treatment approach accordingly.
Both the AIH-PBC and AIH-PSC overlap subtypes are recognised by major liver societies as warranting a combined therapeutic strategy beyond that used for AIH alone.
Treatment Approach
Management of AIH overlap syndrome involves corticosteroid-based immunosuppression combined with a bile acid agent — the specific drugs, dosing, tapering schedule, and full evidence-based algorithm are contained in the complete protocol.
Goal: improvement of liver tests with stabilization of hepatic fibrosis
References
DOI: 10.1002/hep.31065
- Consider adding UDCA to prednisone or prednisolone in combination with AZA in adults and children with AIH and overlap syndromes.
- Prednisone or prednisolone in combination with UDCA has been superior to glucocorticoids alone and UDCA alone in patients satisfying the Paris criteria, and combination therapy has been advocated for patients satisfying the Paris criteria for the AIH-PBC overlap syndrome.
- Prednisone or prednisolone with UDCA has improved survival and reduced frequency of transplantation compared to classical PSC, and this regimen has been advocated by the European and American liver societies for the AIH-PSC overlap syndrome.
- Combination therapy has improved laboratory tests, stabilized hepatic fibrosis, and preserved the 5-year transplant-free (100%) and 10-year overall survival (92%) in patients with AIH-PBC.
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