Primary biliary cirrhosis: geographical clustering and symptomatic onset seasonality.

Abstract

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,

Genes and (auto)immunity in primary biliary cirrhosis

“…Studies from the UK indicate that such frequency ranges between 1 and 2.4%, 8,9 possibly increasing over the past decade, likely for the awareness of physicians and novel diagnostic tools. In the United States, Bach and Schaffner 10 observed a 4.3% familial prevalence in an analysis of 396 PBC cases both retrospectively and prospectively.…”

An exploratory population-based case-control study of primary biliary cirrhosis

“…11 Treatment with various drugs including chlorpromazine, prochlorperazine, and estrogens has also been linked anecdotally with PBC. [12][13][14] Our observation of a large series of PBC patients and published associations with similar diseases suggested that recent surgeries and lifestyle factors, such as smoking, drinking, stressful life events, and pet ownership, would also be worth exploring. Other etiological factors for PBC have been suggested, but we concentrated on those that could be investigated by a self-completion questionnaire.…”

Increased prevalence of primary biliary cirrhosis near superfund toxic waste sites

“…Lastly, several studies have shown that the prevalence of PBC within families of affected individuals is significantly higher than in the general population. 13,16 Differences in the local prevalence of PBC have been reported [7][8][9][10]17,18 and may reflect differences in exposure to environmental toxins. However, strong linkage to any specific environmental factor has not been identified except in a Japanese study 18 showing an increased prevalence of PBC in Hiroshima among atomic bomb survivors.…”

“…5,6 Large geographic variations in the prevalence of PBC have been reported. [7][8][9][10] Differences in both genetic susceptibility factors and exposure to environmental toxins are widely believed to contribute to this variability in the prevalence of PBC.…”