Primary biliary cirrhosis once rare, now common in the United Kingdom?

There is a widespread impression that the number of patients with the autoi mmune liver disease primary biliary cirrhosis (PBC) is increasing, although its incidence and prevalence vary widely. Using thorough case-finding meth ods and rigorous definitions to assess changes in incidence and prevalence with time and to explore the symptomatology and mortality of the disease in a large group of unselected patients, we performed a descriptive epidemiol ogical study of PBC in a well defined population over a fixed period of tim e using established diagnostic criteria and with clinical follow-up of all cases. In a population of 2.05 million in northern England 770 definite or probable PBC cases were identified. Prevalence rose from 201.9 per 10(6) in the adult population and 541.4 per 10(6) women over 40 in 1987 to 334.6 pe r 10(6) adults and 939.8 per 10(6) women over 40 in 1994. Incidence was 23 per 10(6) in 1987 and 32.2 per 10(6) in 1994, Three hundred patients died i n median follow-up of 6.27 years (141 liver deaths); the standardized morta lity ratio was 2.85. At presumed diagnosis, 60.9% had no symptoms of liver disease. By June 1994 62% of prevalent patients had liver symptoms. PBC is apparently increasing. It is still unclear whether this is because of a tru e increase, case finding, or increased disease awareness. The study draws a ttention to (1) high mortality from liver disease and non-liver-related cau ses even in patients initially with no liver symptoms and (2) apparently po or diagnostic awareness of the disease.