What is the treatment for biliary cirrhosis diagnosed on scans?

Treatment for Biliary Cirrhosis Diagnosed on Scans

Ursodeoxycholic acid (UDCA) at a dose of 13-15 mg/kg/day is the first-line treatment for primary biliary cirrhosis, as it significantly improves liver biochemistry, delays histological progression, and reduces the likelihood of liver transplantation or death. 1, 2

First-Line Therapy

• UDCA at 13-15 mg/kg/day is the established treatment of choice for primary biliary cirrhosis (PBC) based on multiple placebo-controlled trials and long-term case-control studies 1
• UDCA significantly decreases serum bilirubin, alkaline phosphatase, cholesterol, and immunoglobulin M levels in PBC patients compared to placebo 2
• Long-term UDCA treatment delays histological progression of PBC when started at an early stage of the disease 3
• UDCA treatment is associated with a significant reduction in the likelihood of liver transplantation or death in patients with moderate to severe PBC 1

Dosing Considerations

• Lower doses (5-7 mg/kg/day) are less effective than standard doses (13-15 mg/kg/day) in improving liver biochemistry 4
• Higher doses (23-25 mg/kg/day) do not provide additional benefits over standard doses but are still well-tolerated 4
• UDCA should be started promptly after diagnosis to maximize benefits, as it is more effective in early-stage disease 5

Monitoring Response to Treatment

• Regular monitoring of liver biochemistry is essential to assess treatment response 1
• Improvement in liver biochemistry should be evaluated after 1 year of UDCA therapy to identify patients at risk of progressive disease 1
• UDCA generally improves serum liver enzymes within one month of starting treatment 5
• Despite biochemical improvement, UDCA may not significantly improve symptoms like fatigue or pruritus in all patients 1, 6

Treatment for Incomplete Responders

• For patients with an inadequate response to UDCA, obeticholic acid (OCALIVA) may be added as a second-line therapy 7
• OCALIVA is indicated for adult PBC patients without cirrhosis or with compensated cirrhosis who do not have evidence of portal hypertension, either in combination with UDCA with an inadequate response to UDCA or as monotherapy in patients unable to tolerate UDCA 7
• Starting dose of OCALIVA is 5 mg once daily for the first 3 months, with potential increase to 10 mg once daily if inadequate response and good tolerability 7

Important Safety Considerations

• OCALIVA is contraindicated in PBC patients with decompensated cirrhosis, a prior decompensation event, or with compensated cirrhosis who have evidence of portal hypertension 7
• Hepatic decompensation and failure, sometimes fatal or resulting in liver transplantation, have been reported with OCALIVA treatment 7
• UDCA is generally well-tolerated, with minimal side effects even at higher doses 4
• UDCA is considered safe during pregnancy and breastfeeding for women with PBC who require continued treatment 1

Management of Complications

• For pruritus, treatment options include rifampin, bile acid binding agents, or opioid antagonists 2
• Sertraline may be considered as a first-line treatment for cholestatic pruritus 2
• Regular assessment of bone health and vitamin D supplementation should be considered 8
• Monitor for development of portal hypertension in patients with compensated cirrhosis 7

Prognosis and Long-term Outcomes

• Early treatment with UDCA significantly delays progression to cirrhosis (13% vs 49% in untreated patients over 5-9 years) 3
• The improvement in liver biochemistry with UDCA treatment does not always correlate with histological improvement 6
• UDCA therapy should be continued long-term as benefits increase with duration of treatment 3