PBC Canada Diagnosis and Treatment







Care pathway information

  • Primary Biliary Cholangitis is a rare autoimmune disease which can lead to liver fibrosis, cirrhosis and endstage liver disease when it’s not treated.
  • The goal of the care pathway is to provide a simpler framework that can be adapted by physicians depending on their needs.
  • It can also be implemented in a way that would allow care to be integrated, consistent and coordinated.
  • The pathway has been created with evidence and recommendations for patient recommendation.
  • The goal of the PBC care pathway is to ensure that there is a minimum standard of care that is provided to patients living with the condition in order to have the correct clinician, have things done correctly and timely.
  • Guideline adoption can be poor in communities with general gastroenterologists.
  • Thus individuals with PBC can be provided with a high quality of healthcare.

1. Reaching a secure diagnosis

Patients with suspected PBC
  • 9/10 women aged 35-60
  • elevated enzymes
  • fatigue, itchiness, jaundice
  • liver histology
  • AIH
Initial Assessment
  • history, physical examination and abdominal ultrasound
  • serum biochemistry: ALP
  • serology: serum AMA and/or PBC-specific ANA
Establish a secure diagnosis
Other possible conditions
  • rule out
  • presence of other conditions

Further imaging tests or biopsies  

2. UDCA therapy, baseline clinical assessment and risk stratification

Initiate 1st Line Treatment
  •  
Perform baseline clinical assessment
  •  
Assess pre-treatment disease stage and long term risk of disease progression
Monitor symptoms
  • fatigue, pruritus, sicca
  •  

Monitor symptom management medications 

3. Response to 1st-line therapy

Assess response to UDCA treatment within 6-12 months

Assess risk of progression based on response to treatment

Reaction to 1st line therapy
    • side effects
    • symptom changes



 

4. On-treatment risk stratification

Stratify risk: low, intermediate, high
  • consider referral for further assessment

Continue UDCA and assess response every 12 months


Further assessment to evaluate risk-benefit of 2nd line treatment
    • further assessment of overall health

5. 2nd-line treatment

Initiate 2nd line therapy in consultation with patient

Treatment assessment and regular follow up


Clinical trials


Reaction/side effectds of 2nd line treatment