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Editorial
. 2022 Feb;16(1):1-23.
doi: 10.1007/s12072-021-10276-6. Epub 2022 Feb 4.

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

Hong You #  1 , Xiong Ma #  2 , Cumali Efe #  3 , Guiqiang Wang  4 , Sook-Hyang Jeong  5 , Kazumichi Abe  6 , Weijia Duan  1 , Sha Chen  1 , Yuanyuan Kong  7 , Dong Zhang  8 , Lai Wei  9 , Fu-Sheng Wang  10 , Han-Chieh Lin  11 , Jin Mo Yang  12 , Tawesak Tanwandee  13 , Rino A Gani  14 , Diana A Payawal  15 , Barjesh C Sharma  16 , Jinlin Hou  17 , Osamu Yokosuka  18 , A Kadir Dokmeci  19 , Darrell Crawford  20 , Jia-Horng Kao  21 , Teerha Piratvisuth  22 , Dong Jin Suh  23 , Laurentius A Lesmana  24 , Jose Sollano  25 , George Lau  26 , Shiv K Sarin  27 , Masao Omata  28   29 , Atsushi Tanaka  30 , Jidong Jia  31
Affiliations
Editorial

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

Hong You et al. Hepatol Int. 2022 Feb.
No abstract available

PubMed Disclaimer

Conflict of interest statement

This guidance were independently prepared and peer reviewed, and all contributors disclosed their conflicts of interest. Hong You, Xiong Ma, Cumali Efe, Guiqiang Wang, Sook‑Hyang Jeong, Kazumichi Abe, Weijia Duan,Sha Chen, Yuanyuan Kong, Dong Zhang, Lai Wei, Fu‑Sheng Wang, Han‑Chieh Lin, Jin Mo Yang,Tawesak Tanwandee, Rino A. Gani, Diana A. Payawal, Barjesh C. Sharma, Jinlin Hou,Osamu Yokosuka, A. Kadir Dokmeci, Darrell Crawford, Jia‑Horng Kao, Teerha Piratvisuth,Dong Jin Suh, Laurentius A. Lesmana, Jose Sollano, George Lau, Shiv K. Sarin, Masao Omata, Atsushi Tanaka, Jidong Jia these authors have nothing to disclose.

Figures

Fig. 1
Fig. 1
Pathogenesis of primary biliary cholangitis. PBC is complex and is thought to be caused by the interplay of genetic (A) and environmental factors (B, C). Exposure to PDC-E2 initiates innate and adaptive immune responses that target biliary epithelial cells and cause inflammation. D Injured cholangiocytes with dysfunctional anion exchanger 2 (AE2) are sensitive to apoptosis and senescence, ultimately leading to cholestasis and liver fibrosis. HLA human leucocyte antigen, miR-506 microRNA 506, miR-21 microRNA 21, PDC-E2 the E2 component of the mitochondrial pyruvate dehydrogenase complex, CXCR3 C-X-C motif chemokine receptor 3, APC antigen presenting cell, IFN-γ interferon-γ, Tfh follicular helper T cell, AMA anti-mitochondrial autoantibody, TGF-β transforming growth factor-β, Treg regulatory T cells, DNT double negative T cell, CTL cytotoxic T lymphocyte, FasL Fas ligand,  MDSC myeloid-derived suppressor cell, CCN1 cellular communication network factor 1, MAIT mucosal-associated invariant T cell, TNF-α tumor necrosis factor-α, NK natural killer, CXCL9 C-X-C motif chemokine ligand 9, CXCL10 C-X-C motif chemokine ligand 10, CCL20 C-C motif chemokine ligand 20, sAC soluble adenylyl cyclase, AE2 anion exchanger 2
Fig. 2
Fig. 2
Diagnostic flowchart for PBC. PBC primary biliary cholangitis, ALP alkaline phosphatase, GGT gamma glutamyl transferase, US ultrasonography, PSC primary sclerosing cholangitis, AMA anti-mitochondrial autoantibody, DILI drug induced liver injury
Fig. 3
Fig. 3
Typical histological features of PBC in different stages. a Stage I: chronic non-suppurative destructive cholangitis (arrow, H&E, 200×). b Stage II: ductular reaction with periportal necroinflammotory activity (H&E, 200×). c Stage III: multiple portal-portal bridging fibrosis (Trichrome, 40×). d Stage IV: biliary cirrhosis with nodule formation (Trichrome, 40×)
Fig. 4
Fig. 4
Risk-based approach for PBC patients. UDCA ursodeoxycholic acids, OCA Obeticholic acid
See this image and copyright information in PMC

References

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