SUMMARY Patients with primary biliary cirrhosis (primary non-suppurative destructive cholangitis) in the north east region of England were studied over a five year period and, to evaluate epidemicity, compared with two contemporaneous disease series of known occurrence. These were: terminal renal failure, all causes (low or absent epidemicity n= 106) and an outbreak of echovirus 19 disease (high epidemicity n=201). Eight primary biliary cirrhosis-affected men and 109 women from an estimated catchment population of 2.08 million were identified. The current diagnosis rate was 1.0/100 000 (1-8/100 000 for women of 15 or more). There were 18 deaths, mean survival from diagnosis 4.0 years. Within the region prevalence varied from 3.7/100 000 in rural areas to 14-4/100 000 in industrial urban areas. In the conurbation, prevalence rates varied insignificantly. Here, most cases were concentrated in central districts, where the proportion of asymptomatic presentations was 50%. Outside the conurbation the asymptomatic proportion fell to 21%, suggesting low incidental diagnosis rates. When compared with echovirus 19, primary biliary cirrhosis was of low or absent epidemicity, and similar to renal failure in its uniform geographical distribution and lack of clustering. Forty three patients (37% of the total), however, had significantly seasonal symptomatic presentations (p<0.01), although scan statistic testing failed to show clustering of onset in time. Apparently provocative factors associated with primary biliary cirrhosis symptomatic onset were identified in only 11 (9.4%) of patients. Age-specific onset rates rose linearly between ages 35 and 65, and nearly one third of patients presented after 65 years, two thirds of deaths occurring in this age group. There is thus no evidence in north east England of geographical anomalies in the distribution of primary biliary cirrhosis. International differences may be partly explained by environmental factors influencing seasonal presentation, such as sunlight. Diagnosis rates are profoundly influenced by increased medical awareness, especially in the elderly, of this now relatively common disease and increased use of the mitrochondrial (AMA) antibody test.Primary biliary cirrhosis (primary non-suppurative are unknown and little is known of its distribution destructive cholangitis) chiefly affects middle aged and occurrence in the general population. This or elderly women and is characterised by paper describes some epidemiological attributes of a progressive destruction of intrahepatic bile ducts. series of patients diagnosed in Newcastle-upon-Tyne After a variable period of increasing cholestasis and its surrounding region. biliary cirrhosis develops, resulting finally in death from hepatic failure or the consequences of portal Methods hypertension.'1 2 The causation of primary biliary cirrhosis and the reason for its female predilection PATIENTS AND CONTROLSThe study was centred upon Newcastle-upon-Tyne,

SUMMARY Primary biliary cirrhosis is a rare disease in the general population. Estimates of its true incidence are difficult but since survival time is unaffected by treatment, mortality may reflect important regional and other variations. One hundred and sixty-five death certificates collected in England and Wales over the five-year period 1967-1971 were inspected and confirmed an overwhelming predilection for females. Deaths rose sharply at ages 50-54 in the latter with a peak of 4.1 million-' year-', with perhaps a secondary peak at ages 70-74. No relation of mortality with climate, altitude, soil type, annual temperature range, or occupation was found, although outside the UK a broad correlation exists with total cirrhosis deaths. There was a suggestive excess of deaths among married women. The greater frequency of deaths in the London area, a rise in mortality from country to urban areas, a fall-off in deaths from primary biliary cirrhosis in old age, and predominance for social class I suggest a simple relationship with standards of medical care or diagnosis.

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BRITISH MEDICAL JOURNAL 26 MARCH 1977 817 been a total of 19 cases in seven families reported, our patients bringing the total to 21 cases in eight families. The familial incidence of hypernephromas may be fortuitous, but if a genetic factor is present the question of screening other members of the family arises. Brinton2 and Steinberg et al3 reported hypernephroma in two generations and it would therefore seem reasonable to carry out regular five-year investigations in the other siblings and the children of our two brothers.We thank Professor N C Nevin for his help, and Mrs D Cranston for typing the manuscript.