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Cited by 133Liver Diseases and ImmunityPediatric Hepatobiliary Diseases and TreatmentsIgG4-Related and Inflammatory Diseases

APASL clinical practice guidance: the diagnosis and management of patients with primary biliary cholangitis

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You, Hong, Ma, Xiong, Efe, Cumali, Wang, Guiqiang, Jeong, Sook-Hyang, Abe, Kazumichi, Duan, Weijia, Chen, Sha +24 more·2022·Hepatology International
DOI 10.1007/s12072-021-10276-6PMC PMC8843914
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Abstract

Primary biliary cholangitis (PBC) is a chronic intrahepatic cholestatic disease with not fully elucidated pathogenesis. Immunological dysfunction triggered by environmental factors may render autoimmunity against the interlobular bile ducts in genetically predisposed hosts. PBC typically affects middle-aged women, commonly presents with fatigue and pruritus, or with an asymptomatic elevation of serum alkaline phosphatase (ALP)/glutamyl transpeptidase (GGT). The pathological features are progressive, non-suppurative, destructive intrahepatic cholangitis, leading to fibrosis and eventually cirrhosis. Antimitochondrial antibodies (AMAs), especially the M2 subtype (AMA-M2), are highly sensitive and specific for PBC in clinical settings. Currently, ursodeoxycholic acid (UDCA) is the treatment of choice for this disease.

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  • A diagnosis of primary biliary cholangitis is made if patients meet at least two of the following criteria: have biochemical evidence of chronic cholestasis, positive anti-mitochondrial antibodies in sera, and histological features compatible with primary biliary cholangitis.

    “A diagnosis of primary biliary cholangitis is made if patients meet at least two of the following criteria: have biochemical evidence of chronic cholestasis, positive anti-mitochondrial antibodies in sera, and histological features compatible with primary biliary cholangitis.[3][4][5]”

    From Primary biliary cholangitis. (2024) · cites this paper as [5]

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  • Clinically, primary biliary cholangitis is characterised by persistent elevation of cholestatic liver enzymes, including alkaline phosphatase and GGT, and detection of antimitochondrial antibodies or PBC-specific anti-nuclear antibodies (anti-sp100 or anti-gp210).

    “Clinically, primary biliary cholangitis is characterised by persistent elevation of cholestatic liver enzymes, including alkaline phosphatase and gammaglutamyl transpeptidase (GGT), and detection of antimitochondrial antibodies or primary biliary cholangitis-specific anti-nuclear antibodies (anti-sp100 or anti-gp210).[3][4][5]”

    From Primary biliary cholangitis. (2024) · cites this paper as [5]

  • Clinical practice guidelines from EASL, AASLD, and APASL recommend ursodeoxycholic acid treatment (13-15 mg/kg per day) for all patients with primary biliary cholangitis and elevated cholestatic enzymes.

    “Clinical practice guidelines from the European Association for the Study of the Liver, the American Association for the Study of Liver Diseases (AASLD), and the Asian-Pacific Association for the Study of the Liver recommend ursodeoxycholic acid treatment (13-15 mg/kg per day) for all patients with primary biliary cholangitis and elevated cholestatic enzymes.[3][4][5]”

    From Primary biliary cholangitis. (2024) · cites this paper as [5]

  • Ursodeoxycholic acid is standard-of-care treatment for primary biliary cholangitis.

    “Ursodeoxycholic acid is standard-of-care treatment for primary biliary cholangitis.”

    From Primary biliary cholangitis. (2024) · cites this paper as [5]

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Authors
You, H., Ma, X., Efe, C., Wang, G., Jeong, S.-H., Abe, K., Duan, W., Chen, S., Kong, Y., Zhang, D., Wei, L., Wang, F.-S., Lin, H.-C., Yang, J. M., Tanwandee, T., Gani, R. A., Payawal, D. A., Sharma, B. C., Hou, J., Yokosuka, O., Dokmeci, A. K., Crawford, D., Kao, J.-H., Piratvisuth, T., Suh, D. J., Lesmana, L. A., Sollano, J., Lau, G., Sarin, S. K., Omata, M., Tanaka, A., & Jia, J.
Affiliations
  • Capital Medical University (CN)
  • Shanghai Jiao Tong University (CN)
  • Diyarbakır Gazi Yaşargil Eğitim ve Araştırma Hastanesi (TR)
  • Peking University First Hospital (CN)
  • Seoul National University (KR)
  • Fukushima Medical University (JP)
  • Beijing Tsinghua Chang Gung Hospital (CN)
  • Chinese PLA General Hospital (CN)
  • Taipei Veterans General Hospital (TW)
  • The Catholic University of Korea St. Vincent's Hospital (KR)
  • Siriraj Hospital (TH)
  • University of Indonesia (ID)
  • Govind Ballabh Pant Hospital (IN)
  • Nanfang Hospital (CN)
  • Chiba University (JP)
  • National Taiwan University Hospital (TW)
  • Prince of Songkla University (TH)
  • University of Ulsan (KR)
  • University of Santo Tomas (PH)
  • Humanity & Health (CN)
  • Institute of Liver and Biliary Sciences (IN)
  • Yamanashi Prefectural Central Hospital (JP)
  • Teikyo University (JP)
Corresponding author
  • Omata, Masao
  • Tanaka, Atsushi
  • Jia, Jidong
ORCID
Venue
Hepatology International
Publisher
Springer Science and Business Media LLC
Published
2022-02
Volume
16
Issue
1
Pages
1-23
ISSN
  • 1936-0533 (ISSN-L)
  • 1936-0541
DOI
10.1007/s12072-021-10276-6
PMC
PMC8843914
OpenAlex
W4210540808
Cited by
133
References
239
Citations by year
2026: 30 · 2025: 31 · 2024: 38 · 2023: 26 · 2022: 8
FWCI
15.35
Type
journal article
Language
en
Open access
Licence
Locations
  • Hepatology International (publishedVersion, cc-by)
  • PubMed Central (publishedVersion, cc-by)
  • PubMed (publishedVersion)
  • Institutional Repositories DataBase (IRDB) (submittedVersion, cc-by-nc-nd)
Funders
  • Capital Medical University
  • Shanghai Jiao Tong University
  • Renji Hospital
  • Beijing Friendship Hospital, Capital Medical University
Topics
  • Health Sciences › Medicine › Hepatology › Liver Diseases and Immunity
  • Health Sciences › Medicine › Surgery › Pediatric Hepatobiliary Diseases and Treatments
  • Health Sciences › Medicine › Rheumatology › IgG4-Related and Inflammatory Diseases
Keywords
primary biliary cirrhosis, intrahepatic cholestasis, antimitochondrial antibodies, AMA-M2, ursodeoxycholic acid, cholestatic liver disease, liver fibrosis, liver cirrhosis, autoimmune liver disease, diagnosis, management
MeSH terms
Cholangitis, Humans, Liver Cirrhosis, Biliary

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