Pathogenesis of primary biliary cirrhosis

3 4 Early descriptions of PBC emphasised the predominant role played by the progression of bile duct loss, accompanied by increasing portal tract and linking fibrosis leading to biliary cirrhosis, in the clinical expression of PBC, and described an aggressive and uniformly fatal condition. 5 Increased awareness of the condition and, in particular, the availability of diagnostic tools such as serology has led to broader and earlier diagnosis. 6 This has had the effect that we now recognise PBC far more frequently than was previously the case, and typically do so at an earlier stage in the disease process. The identical population frequency reflects the fact that these are, de facto, the same antibodies. [dagger]Primary biliary cirrhosis population frequencies to these antibodies appear to show significant geographical variation and this is reflected in the varying reported frequencies. [double dagger]2-Oxoglutarate dehydrogenase (OGDC)-E2, branched chain 2-oxoacid dehydroganese (BCOADC)-E2 and PDC-E3BP share an apparent molecular weight on sodium dodecylsulphate-polyacrylamide gel electrophoresis in the range 50-52 kDa (with subtle intermammalian species differences that can be important depending on the source of the PDC used in immunoblots).