Variant forms of PBC
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The PBC Foundation is the only UK organisation exclusively dedicated to providing support and information to those affected by PBC

Variant forms of PBC

Variant forms of PBC

AMA-negative primary biliary cholangitis

Approximately 5% of PBC patients have AMA-negative PBC, meaning that clinical, biochemical and histological features are characteristic of PBC but AMA cannot be detected in the serum.  The clinical manifestations and natural history of AMA-negative and AMA-positive PBC appear to be the same.  However, AMA-negative PBC has not been well-characterised because the condition is rare.

Premature ductopenic variant

The premature ductopenic variant is a form of PBC characterised histologically by extreme ductopenia that is disproportionate to the extent of liver fibrosis.  Owing to markedly decreased quality of life and the adverse effect of chronic severe cholestasis on nutritional status, liver transplantation is generally required within a few years of presentation.

PBC/AIH overlap syndrome

The term ‘AIH/PBC overlap syndrome’ is used to describe variant forms of PBC in which there are characteristics of both AIH and PBC.  In approximately 8-10% of patients with a diagnosis of PBC, features of AIH coexist. In addition to AIH/PBC overlap (in which patients have features of both disorders), it has also been reported that patients may present with typical picture of PBC, which then evolves into a typical picture of AIH over a time-frame of 6 months to 13 years.