APASL clinical practice guidance: the diagnosis and management of patients with primary biliary cholangitis
APASL · 2022 · international
Source and licence
- Authors
- Hong You, Xiong Ma, Cumali Efe et al.
- Title
- APASL clinical practice guidance: the diagnosis and management of patients with primary biliary cholangitis
- Journal
- Hepatology International, 2022
- Licence
- CC BY 4.0
- Changes
- Verbatim excerpts; formatting adapted
Full text retrieved 2026-10-27 from Europe PMC · PMC8843914
Licence statement of the original publication
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This page reproduces excerpts of the original publication; the original is authoritative. It has not been reviewed by a physician, does not state a recommendation of Medelix and does not replace medical advice. The guideline may have been updated since.
Open the original publication →Full text on Europe PMC
Recommendations
Verbatim, with grade of recommendation or strength of consensus where given.
Diagnosis criteria
- Recommendations1. The diagnosis of PBC can be established when meeting two or more of the following three criteria: (I, 1) Biochemical evidence of cholestasis based mainly on the elevation of ALP and GGT with the exclusion of extrahepatic biliary obstruction by imaging studies; Presence of AMA or other PBC-specific ANAs including anti-sp100 or anti-gp210; Histologic evidence of non-suppurative destructive cholangitis mainly affecting the interlobular bile ducts.
Lifestyle modification
- Recommendation2. In the context of the negative impacts of cigarette smoking, alcohol intake, and obesity on human health, PBC patients should be encouraged to quit smoking, stop alcohol drinking, and keep on ideal body weight. (III, 2).
First-line treatment: UDCA
- Recommendation3. It is recommended that oral UDCA (13 ~ 15 mg/kg/day) should be standard therapy for all PBC patients. UDCA treatment should be continued for prolonged periods, and compliance to therapy should be checked (I, 1).
Criteria of biochemical response to UDCA
- Recommendation4. It is recommended that evaluating all PBC patients for the biochemical response with appropriate criteria after 6 months of UDCA treatment (III, 1), or 12 months of UDCA treatment (II, 1)
Obeticholic acid
- Recommendation5. It is recommended that OCA (starting at 5 mg/day, increasing to 10 mg/day after 6 months if tolerated well) be added to UDCA therapy for PBC patients (non-cirrhotic or cirrhosis with Child–Pugh–Turcotte A) and an inadequate response to UDCA or used as monotherapy in those intolerant to UDCA. Potential risks and adverse events of OCA should be discussed in detail with the patient, carefully evaluated and appropriately monitored. (I, 1)
Fibrates (Fenofibrate and Bezafibrate)
- Recommendation6. It is recommended that bezafibrate (400 mg/day) or fenofibrate (200 mg/day) could be added to UDCA for patients with an inadequate response to UDCA monotherapy. Adverse events should be closely monitored, especially in cirrhotic PBC patients. (I, 1).
Budesonide
- Recommendation7. Budesonide (6–9 mg/day) might be added to non-cirrhotic PBC patients with suboptimal response to UDCA. (II, 2)
Pruritus
- Recommendations8. It is recommended that cholestyramine (4–16 g/day) as the first-line therapy for pruritus. To avoid interference with the absorption, take other medications including UDCA at least 1 h before or 4 to 6 h after taking cholestyramine. (II, 2)
Vitamin D deficiency and osteoporosis
- Recommendations10. It is recommended that all PBC patients should be evaluated for serum vitamin D status. (II, 2)
Fatigue
- Recommendation15. No specific medical therapy is available for fatigue. Treating co-existent conditions such as anemia, extra-hepatic autoimmune disease, sleep disturbance, and depression are recommended to manage fatigue. (III, 2)
Portal hypertension
- Recommendation16. Patients with features of portal hypertention (ie. splenomegaly, thrombocytopenia) should be screened for gastroesophageal varices. (II, 2)
Hepatocellular carcinoma
- Recommendation17. Close monitoring of HCC is strongly recommended for males, patients with advanced-stage disease, and non-responders to UDCA. (II, 2)
Liver transplantation
- Recommendations18. It is recommended that liver transplantation should be considered in patients with decompensated cirrhosis, MELD ≥ 15, Mayo Risk Score > 7.8, or severe, intractable pruritus. (II, 1)
PBC with AIH features (formerly known as PBC-AIH overlap syndrome)
- Recommendations20. The diagnosis of PBC with AIH features could be made in PBC patients if two of the three following criteria are met: (1) moderate/severe interface hepatitis in liver histology (mandatory); (2) serum ALT/AST more than 5 times ULN; and (3) IgG level more than 1.3 times ULN or the presence of ASMA. (III, 2)
AMA-negative PBC
- Recommendations22. It is recommended that liver biopsy should be performed on patients who present otherwise unexplained cholestatic liver biochemistry and negative for AMA, anti-gp210, or anti-sp100 to confirm the diagnosis of PBC. (III, 2)
Isolated AMA positivity
- Recommendation23. AMA reactivity alone is not sufficient to diagnose PBC. AMA-positive patients with normal serum liver tests should be followed up with an annual biochemical reassessment for the presence of liver disease. A liver biopsy may be considered in selected patients (eg. patients with elevated IgM, high titer of AMA, or ALP close to ULN) to identify preclinical PBC. (III, 2)
Pregnancy
- Recommendations24. Pregnancy can be advised in PBC patients at childbearing age. Patients with features of cirrhosis should be well informed about the possible maternal and fetal complications. (III, 2)
Contents of the original publication
The section headings of the original.
- Introduction
- Guidance development process
- Epidemiology
- Pathogenesis
- Diagnosis of PBC
- Clinical features
- Laboratory tests
- Imaging examinations
- Pathological characteristics and histological staging
- Diagnosis criteria
- Differential diagnosis
- Management of PBC
- Lifestyle modification
- First-line treatment: UDCA
- Criteria of biochemical response to UDCA
- Second-line therapy for suboptimal responders to UDCA
- Management of symptoms and complications
- Hepatocellular carcinoma
- Liver transplantation
- Special conditions
- PBC with AIH features (formerly known as PBC-AIH overlap syndrome)
- AMA-negative PBC
- Isolated AMA positivity
- Pregnancy
- Natural history and prognosis
- Natural history and clinical course
- Risk factors for poor clinical outcomes
- Future research and horizons
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