Faculty Bibliography

The epidemiology of primary biliary cirrhosis was described as early as the 1970s, yet decades later the true frequency of this disease and its associated risk factors are still in question. There has been a wealth of data documenting the various incidence and prevalence rates across the world, demonstrating potential risk factors inherent to geographic differences. Studies that follow primary biliary cirrhosis in a set population over time have offered the most reliable picture of disease frequency. Analysis of clustering effects through region and time has offered valuable information on the complexity of the disease development. Improved epidemiologic surveillance of primary biliary cirrhosis around the world will be necessary to provide definitive evidence on the phenomenon of clustering and its associations with proposed risk factors in the literature

Since initial reports in the mid-1970s provided epidemiology data on primary biliary cirrhosis (PBC), many studies have characterized the variable frequency of this disease in diverse populations worldwide and sought to identify associated risk factors. Recent research confirms earlier work suggesting that the incidence and prevalence of PBC are on the rise, although geographic variation persists. Analysis of familial and geographic clustering supports the hypothesis that development and progression of the disease hinge on a complex interplay between genetic and environmental risk factors. International clinical data systems are needed to advance PBC epidemiologic research. Given this complexity, international clinical data systems are needed to advance PBC epidemiologic research