The PBC Foundation is the only UK organisation exclusively dedicated to providing support and information to those affected by PBC
Management of PBC
There is no cure for PBC. General health measures are important. Medications for PBC may be classified as disease-modifying or symptom-modifying. Patients with ESLD should be considered for liver transplantation.
General health measures
Tobacco smoking has been shown to increase the risk of liver fibrosis in PBC therefore patients should be strongly encouraged not to smoke. There is probably no specific benefit from avoiding alcohol altogether but consumption should not exceed recommended levels, i.e. men should not regularly drink more than 3 – 4 units of alcohol per day and women should not regularly consume more than 2 – 3 units per day. Patients should consume a healthful diet; engage in regular exercise, and maintain a normal body mass index (BMI).
The only treatment licensed and used routinely for disease-modifying treatment of PBC is ursodeoxycholic acid (UDCA). Numerous other medications have been tried but are not used, either because they are ineffective in PBC or because they have side-effects that outweigh their potential benefits.
UDCA is a bile acid that is normally present in human bile at low concentrations. Studies suggest that UDCA brings about improvement in the liver biochemistry; slows the rate of histological progression, and improves LT-free survival. Guidelines from the European Association for the Study of the Liver suggest that all patients with PBC should be treated with UDCA, 13 – 15 mg/kg/day. UDCA is generally well-tolerated although patients may experience weight gain or gastro-intestinal symptoms such as abdominal discomfort, increased bowel frequency or constipation. The biochemical response to UDCA should be evaluated 12 months after starting treatment to guide further care. UDCA non-responders may require specialist care. There are no well-established second-line therapies but small studies suggest that UDCA non-responders might benefit from fibrates or the combination of mycophenolate and budesonide. Some novel therapies that show promise but are still under evaluation.
PBC is a well-established indication for liver transplantation (LT) and LT is an excellent treatment for PBC-related liver failure. LT may also be indicated for severe, refractory pruritus. PBC recurs after LT and recurrent PBC may be found in some 25% of liver allografts at median 6.5 years post-LT. However, recurrent PBC very rarely causes graft failure.