Pathway AI

Account ⋅ Sign Out

Table of contents

Primary biliary cholangitis

Key sources
The following summarized guidelines for the evaluation and management of primary biliary cholangitis are prepared by our editorial team based on guidelines from the Asian Pacific Association for the Study of the Liver (APASL 2022), the American Association for the Study of Liver Diseases (AASLD 2021; 2019), the British Society of Gastroenterology (BSG 2018), the American College of Gastroenterology (ACG 2016), and the Italian Association for the Study of the Liver (AISF 2016).


1.Screening and diagnosis

Indications for screening: as per BSG 2018 guidelines, do not obtain routine screening for PBC in relatives of patients with PBC.
Create free account

More topics in this section

  • Diagnostic criteria

2.Classification and risk stratification

Risk assessment: evaluate disease severity and activity at baseline and on treatment during risk assessment of patients with PBC. Consider using the combination of the following to aid risk stratification:
serum liver tests (to identify patients with an elevated bilirubin, a platelet count < 150 or biochemical disease activity on treatment)
imaging (liver ultrasound to identify overt cirrhosis and splenomegaly, transient elastography to identify increased liver stiffness)
recognition of young age at disease onset (< 45 years) and male sex

3.Diagnostic investigations

History and physical examination: evaluate all patients for the presence of symptoms, particularly fatigue and itch, recognizing that the severity of symptoms does not correlate with stage of disease.

More topics in this section

  • Laboratory tests

  • Diagnostic imaging

  • Evaluation for osteoporosis

  • Screening for comorbidities

4.Diagnostic procedures

Upper gastrointestinal endoscopy: as per APASL 2022 guidelines, perform screen endoscopy for gastroesophageal varices in patients with features of portal hypertension (splenomegaly, thrombocytopenia).

More topics in this section

  • Liver biopsy

5.Medical management

Setting of care: consider providing hospital-led care in patients with any of the following:
UDCA non-responsive disease
advanced liver fibrosis/cirrhosis
features of portal hypertension
complex symptoms
Show 2 more

More topics in this section

  • First-line therapy

  • Second-line therapy

  • Management of pruritus

  • Management of fatigue

  • Management of sicca symptoms

  • Management of vitamin deficiencies

  • Management of osteoporosis

  • Management of ascites and hepatic encephalopathy

  • Management of dyslipidemia

6.Nonpharmacologic interventions

Lifestyle modifications: encourage patients with PBC to quit smoking, stop alcohol drinking and keep on ideal body weight because of the negative impacts of these factors on human health.

More topics in this section

  • Calcium and vitamin D supplementation

  • Psychosocial support

7.Surgical interventions

Liver transplantation
As per APASL 2022 guidelines:
Consider performing liver transplantation in patients with any of the following:
decompensated cirrhosis
MELD ≥ 15
mayo Risk Score > 7.8
severe, intractable pruritus
Initiate post-transplant UDCA to improve liver function tests and prevent PBC recurrence.

8.Specific circumstances

Pregnant patients
As per APASL 2022 guidelines:
Counsel patients with PBC at chilebearing age that pregnancy can be attempted. Inform patients with features of cirrhosis about the possible maternal and fetal complications.
Consider continuing UDCA during pregnancy and breastfeeding after special counseling on these particular issues.

More topics in this section

  • Patients with PBC/AIH overlap

9.Follow-up and surveillance

Indications for referral: as per AASLD 2019 guidelines, refer patients with manifestations of end-stage PBC for liver transplantation when their MELD score exceeds 14.

More topics in this section

  • Follow-up

  • Surveillance for HCC

10.Quality improvement

Clinical audit: consider implementing clinical audit tools to document and improve the quality of care delivered to patients with PBC.