A subset of patients with Primary sclerosing cholangitis (PSC) present with biochemical and histological features overlapping with autoimmune hepatitis (AIH). This distinct clinical situation warrants careful evaluation and a specific treatment strategy.
This protocol addresses patients with PSC who show biochemically and histologically suggestive features of autoimmune hepatitis, characterised by:
In this setting, liver biopsy is an important step to confirm histological overlap before committing to a treatment course.
Management involves immunosuppressive therapy under close clinical monitoring — the specific agents, combinations, and sequencing are detailed in the full structured protocol.
DOI: 10.1016/j.jhep.2022.05.011
In people with PSC with biochemically (ALT, IgG, autoantibodies) and histologically suggestive features of AIH, it is suggested to consider corticosteroids or other immunosuppressive therapies under close monitoring.
A liver biopsy should be considered in people with PSC and co-existing features of AIH including markedly elevated transaminases, high IgG levels, and positive autoantibodies compatible with AIH.
We therefore propose considering liver biopsy in people with PSC with alanine aminotransferase (ALT) >5x the upper limit of normal (ULN) and/or IgG level >1.5x ULN.
UDCA in combination with an immunosuppressive regimen (usually prednisolone and azathioprine) is an adequate medical treatment for adults with PSC and features of AIH (PSC-AIH variant syndrome), although no data from controlled clinical trials exist.
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