Primary Biliary Cirrhosis (cont.)
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What is the scope of the problem?
PBC is a disease that disproportionately affects women, with 10 women for
every man having the disease. It is also a disease of adulthood that, rather
curiously, has never been diagnosed in childhood. As a matter of fact, the
diagnosis is made most frequently in middle-aged people, between the ages of
about 30 and 60 years. PBC is considered to be an uncommon disease, but not
rare. Studies indicate that the number of people with PBC at a given time
(referred to as the prevalence of disease) ranges from 19 to 251 per million
population in various countries. If these figures are adjusted to compensate for
the fact that PBC is found only in adults and that 90% of the patients are
women, then the calculated prevalence is approximately 25 to 335 per million
women and 2.8 to 37 per million men.
The largest and best long-term studies of PBC have been conducted in northern
England. Their findings indicate that the number of new cases of PBC over time
(referred to as the incidence of disease) has increased steadily from 16 per
million population in 1976 to 251 per million in 1994. Unfortunately, no similar
studies have been conducted elsewhere to validate or refute the belief that the
incidence and prevalence of PBC is rising worldwide.
One comprehensive study conducted in the north of England from 1987 to 1994
was designed specifically to find people with PBC. Using strict criteria for the
diagnosis of PBC, they identified a total of 770 patients. Of these, the number
of newly diagnosed people with PBC during just these 7 years was 468. Thus,
clinical investigators interested in PBC had conducted extensive epidemiological
(cause and distribution) studies of PBC over almost 20 years in this same
geographic area. Such a concentrated focus of effort strongly supports the view
that the apparent increase in the number of people with PBC is indeed a true
increase.
Next: What is the cause of PBC? »
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