Management of Primary Biliary Cirrhosis
First-Line Treatment
- Ursodeoxycholic acid (UDCA) is the first-line treatment for primary biliary cirrhosis (PBC) at a dose of 13-15 mg/kg/day, with evidence showing it delays histological progression to cirrhosis and improves biochemical markers of cholestasis 1
- UDCA is considered safe during pregnancy and should be continued peri-conception, peri-partum, and post-partum in women of reproductive age 2
Second-Line Therapy
- Obeticholic acid is contraindicated in patients with decompensated cirrhosis, prior decompensation events, or compensated cirrhosis with evidence of portal hypertension 1
Monitoring and Response Assessment
- Routinely monitor patients during treatment for biochemical response, tolerability, and disease progression 1
- Non-responders to treatment who did not have advanced disease at presentation require life-long follow-up with annual monitoring for progression 1
- Patients with mild disease and near-normal liver biochemistry tests require less intensive follow-up with yearly liver function tests 1
- Bilirubin >50 μmol/L is a predictor of adverse outcome; such patients should be discussed with a hepatologist experienced in managing advanced disease 1
Management of Complications
Pruritus
- Add bile acid binding resins or antihistamines for pruritus management 1, 3
- Rifampicin is considered safe for pruritus management during pregnancy 2
- In severe cases during pregnancy, plasmapheresis may be considered 2
Portal Hypertension and Cirrhosis
- Screen for varices according to standard guidelines for cirrhotic patients 1
- Patients with liver stiffness <20 kPa and platelet count >150,000 are at very low risk of having varices requiring treatment 1
- Annual assessment using transient elastography and platelet count should be considered 1
- Manage ascites according to current guidelines for portal hypertension 1
- Consider transjugular intrahepatic portosystemic shunt (TIPS) for patients with variceal bleeding that fails to respond to endoscopic or pharmacological therapy 1
Sicca Complex
- Artificial tears and saliva are often helpful for symptoms of dry eyes and mouth 4
- Pilocarpine or cevimeline can be used for refractory symptoms 4
- Provide oral hygiene advice to prevent dental caries in patients with severe xerostomia 4
- Monitor for oral candidiasis in patients with severe xerostomia 4
Special Populations
Pregnancy
- PBC is typically well-tolerated in non-cirrhotic patients, though pruritus may worsen 2
- Continue UDCA during pregnancy, as expert opinion considers it safe 2
- Pregnant patients with portal hypertension should be electively endoscoped in the second trimester 2
- Screen pregnant PBC patients for anti-Ro and anti-La antibodies, as their presence would affect obstetric management 2
PBC/AIH Overlap Syndrome
- True overlap with autoimmune hepatitis is rare and requires liver biopsy with expert clinicopathological review for diagnosis 5
- Consider liver biopsy in patients with marked hepatitic activity and elevated IgG concentrations 6
- Severe interface hepatitis in the appropriate context may warrant immunosuppressive therapy after discussing risks and benefits with the patient 6
Liver Transplantation
- Consider liver transplantation for patients with bilirubin >50 μmol/L or evidence of decompensated liver disease 1
- Discuss transplantation with a hepatologist linked to a liver transplant program 1
Hepatocellular Carcinoma Screening
- Patients with PBC who have cirrhosis are at increased risk of hepatocellular carcinoma 1
- Non-responders to treatment and male patients are at greater risk 1
- Screening should follow international guidelines for cirrhotic patients 1