The PBC Foundation is the only UK organisation exclusively dedicated to providing support and information to those affected by PBC
PBC for Health Professionals
PBC (Primary Biliary Cholangitis) is an autoimmune liver disease with unknown cause. It is in the group of autoimmune diseases which include thyroid disease, some forms of diabetes mellitus, rheumatoid arthritis and Sjögren’s Syndrome. PBC patients may also have one or more other autoimmune conditions.
In PBC, the immune “attack” is upon smaller intrahepatic bile ducts, this ultimately leads to destruction of the bile ducts - a ‘disappearing bile duct’ syndrome - cholestasis and, in the latter stages of the disease, jaundice.
Like most other autoimmune conditions, PBC affects mostly women (in Britain, about 90% of diagnosed patients). Generally it presents between ages thirty and fifty five although it may be diagnosed at any age in adults and is rarely, if ever, diagnosed in children. There is no connection between PBC and either alcohol or any of the established hepatitis viruses.
Although the cause of PBC is unknown, present theories of aetiology circulate around the hypothesis that molecular mimicry is involved. It is hypothesised that in susceptible individuals - particularly women, possibly with some other genetic susceptibility traits - antibodies are formed against some common, normally non-pathogenic, environmental agent which becomes involved in an immune process pathogenic to the host. These are the antimitochondrial antibodies which, because of molecular mimicry, are directed against host proteins.
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