There is no known effective therapy to prevent the progression of liver disease in PBC. In patients with end-stage liver failure, liver transplantation is “curative,” since recurrence after transplantation is rare. The other therapies listed have been reported as effective in small case series but not in controlled trials.
Ursodeoxycholic acid seems effective in providing at least symptomatic improvement, and may even delay liver transplantation.
Replacement of fat-soluble vitamins (e.g., vitamin A to prevent night blindness) is an important part of therapy, as is replacement of calcium and vitamin D to prevent osteoporosis.