Primary biliary cholangitis (PBC) is a slowly progressive, bile duct disease, which results from chronic inflammation of the small intrahepatic bile ducts, the system of small tubes in the liver that helps drain bile. Those who are diagnosed in early stages and who respond to medication, can have a normal life expectancy.
What is a Care Pathway?
The PBC Integrated Care Pathway is a step-by-step guide for physicians who treat patients with PBC. This pathway is designed to complement the EASL Clinical Practice Guidelines for the diagnosis and management of PBC patients, by providing a decision tree of the key steps in PBC management.
- Research a secure diagnosis of PBC
- UDCA therapy, baseline clinical assessment and risk stratification
- Response to 1st-Line therapy
- On-treatment risk stratification
- 2nd-Line treatment
PBC Care Pathway
1. Research a secure diagnosis of PBC
- Persistent cholestatic abnormalities in serum biochemistry: elevated ALP/GGT/AST/ALT and/or conjugated bilirubin
- Symptoms including pruritus, sicca, arthralgias or fatigue
- Elevated ALP
- AMA-positive (>1/40) or anti-gp210/anti-sp100-positive
2. UDCA therapy, baseline clinical assessment and risk stratification
Key investigations: Blood tests: ALP, bilirubin, AST, ALT, GGT, platelets / Liver ultrasound / Liver stiffness measurement
3. Response to 1st-Line therapy
Assess for evidence of fibrosis/cirrhosis e.g. elastography
Assess risk of progression based on response to treatment
4. On-treatment risk stratification
Many prognostic tools (e.g. Paris-II, Toronto, Barcelona) are used to assess response to first-line therapy; the key markers to measure are ALP and bilirubin, as these are two of the best individual variables that can be used to predict PBC prognosis.
• Patients with low risk of disease progression should be maintained on UDCA, and assessed for their response to UDCA every 12 months.
• All other patients should be considered for second-line treatment only after assessing the benefit to the patient on a case-by-case basis.
5. 2nd-Line treatment
• Patients with an inadequate response to UDCA are at high risk of disease progression,1 and should therefore be moved on to second-line therapy as soon as possible.
• Refer to the OCALIVA Treatment Protocol for further information on licensed second-line therapy.
Off-label therapies (e.g. fibrates, budesonide