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Primary biliary cholangitis

Guidelines

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 World Federation for Ultrasound in Medicine and Biology (WFUMB 2024), 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 ...
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Screening and diagnosis

Indications for screening: as per BSG 2018 guidelines, do not obtain routine screening for PBC in relatives of patients with PBC.
D
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  • Diagnostic criteria

Classification and risk stratification

Risk assessment: as per BSG 2018 guidelines, 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.
B

Diagnostic investigations

History and physical examination: as per BSG 2018 guidelines, 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.
B

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  • Laboratory tests

  • Abdominal ultrasound

  • Liver elastography

  • Evaluation for osteoporosis

  • Screening for comorbidities

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).
B

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  • Liver biopsy

Medical management

Setting of care: as per BSG 2018 guidelines, 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.
C
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  • 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

Nonpharmacologic interventions

Lifestyle modifications: as per APASL 2022 guidelines, 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.
B

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  • Calcium and vitamin D supplementation

  • Psychosocial support

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
B
Initiate post-transplant UDCA to improve liver function tests and prevent PBC recurrence.
B

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.
B
Consider continuing UDCA during pregnancy and breastfeeding after special counseling on these particular issues.
C

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  • Patients with PBC/AIH overlap

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.
E

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  • Follow-up

  • Surveillance for HCC

Quality improvement

Clinical audit: as per BSG 2018 guidelines, consider implementing clinical audit tools to document and improve the quality of care delivered to patients with PBC.
B