It would be improper to proceed with a chapter on primary biliary cirrhosis, without first reflecting upon a great contribution to the knowledge and understanding of the disease made by the late David R. Triger. Unfortunately, his premature and untimely death prevented him from writing this chapter. David Triger had a lifelong zest for the disease, its pathophysiology, clinical management and provided novel observations concerning its epidemiology.
Primary biliary cirrhosis (PBC) is a disease of unknown aetiology associated with a variety of immunological disturbances which suggest a breakdown of immune tolerance. It is characterised by chronic infiltration in the liver and destruction of intrahepatic bile ducts and usually leads to cholestasis, portal hypertension, cirrhosis and death due to liver failure.
Incidence and geography
The disease has been reported from most parts of the world and appears to affect all ethnic populations studied. A study of the prevalence, incidence and death rates in various countries is presently being carried out under the direction of the International Association for the Study of the Liver. Provisional data suggest a marked variation across the world, which probably cannot be adequately explained by differences in clinical practice and diagnostic accuracy alone.
Many series have described incidence and prevalence of PBC in predominantly Caucasian populations. In the UK the incidence is reported to be 5–10/million/year and prevalence of approximately 25–50/million (Triger, 1980; Hamlyn, Macklon & James, 1983).
Email your librarian or administrator to recommend adding this book to your organisation's collection.