Primary biliary cirrhosis (PBC) is a chronic liver disease. It generally strikes women between the ages of 40 and 60, but it has been diagnosed outside of this age range as well as in men. While PBC is not considered to be hereditary, the disease can be seen occasionally in siblings. At the present time, the exact cause is unknown; however, it is known that PBC is not caused by alcohol.
The prevalence of PBC has been estimated as 12.9 per 100,000 population with up to 90% of cases occurring in women. PBC is more common amongst those of northern European descent and less common amongst those of African origin. Diagnosis is usually between about 45 and 55 years old. The prevalence appears to have been rising since 1980.
There are medications that can help slow the progression of the condition and help relieve the itchiness associated with it. In cases where there is extensive liver damage, a liver transplant may be required. Plasmapheresis may be effective in patients with severe pruritus intractable to medical treatment. Dronabinol (Marinol) use is supported by some evidence.