Epidemiology

The prevalence of PBC in the US is approximately 35 per 100,000 of the population overall, with an incidence of approximately 3 to 5 cases per 100,000 per year.[3][4]​​​​​ It is significantly more common in women than in men (up to a tenfold difference); however, men appear to have a poorer prognosis.[4][5][6]​​​​ Studies suggest that at least one in 1000 women over the age of 40 years has PBC.[7]​ Peak incidence is around age 40 years.[7]​ Median age at diagnosis is 65 years.[2]​ Patients can present from their 20s onward and younger patients may experience more aggressive, less treatment-responsive disease.[2]​ There are anecdotal reports only of the disease in children.

Data on ethnic variations are limited, but risk for PBC appears to be broadly similar (although not identical) in different ethnic groups within the same geographical environment (an observation in keeping with an environmental factor being involved in disease pathogenesis). The possibility of the disease should be considered in all ethnic groups.

The incidence and prevalence of PBC in European countries is slightly lower than that in the US, with a prevalence of 22 cases per 100,000, and an annual incidence of 1 to 2 new cases per 100,000 people.[8]​ In both Europe and the US the prevalence of PBC is increasing, which may be due to earlier diagnosis and decreased mortality due to treatment with ursodiol.[9]​ The disease is substantially less common (although not well studied) in African and Asian countries, compared with Europe and the US. However, the incidence of the disease has been widely reported to be increasing in China; one meta-analysis of PBC in the Asia-Pacific region showed the prevalence was 19 per 100,000 in Japan and China.[10][11][12]​​​​

PBC is associated with immune-system and metabolic disorders, including thyroid disease, scleroderma, Sjögren syndrome/sicca complex (dry eyes and mouth), celiac disease, and osteoporosis.[13][14][15]​​ In one controlled interview-based study of 1032 patients, autoimmune diseases were found in 32% of PBC cases, compared with 13% of controls (P <0.0001).[13]

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