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

You, Hong; Ma, Xiong; Efe, Cumali; Wang, Guiqiang; Jeong, Sook‐Hyang; Abe, Kazumichi; Duan, Weijia; Chen, Sha; Kong, Yuanyuan; Zhang, Dong; Wei, Lai; Wang, Fusheng; H, Lin; Yang, Jin Mo; Tanwandee, Tawesak; Gani, Rino Alvani; Payawal, Diana A.; Sharma, Barjesh C.; Hou, Jinlin; Yokosuka, Osamu; Dokmeci, A. Kadir; Crawford, Darrell H. G.; Kao, Jia‐Horng; Piratvisuth, Teerha; Suh, Dong Jin; Lesmana, Laurentius A.; Sollano, José D.; Lau, George; Sarin, Shiv Kumar; Omata, Masao; Tanaka, Atsushi; Jia, Jidong

doi:10.1007/s12072-021-10276-6

Cited by 61 publications

(88 citation statements)

References 239 publications

(306 reference statements)

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“…UDCA at a dose of 13–15 mg/kg/day has been recommended to decrease the progression of PBC [ 102 , 106 ]. To define the biochemical response, several criteria have been proposed ( Table 1 ).…”

Section: Primary Biliary Cholangitismentioning

confidence: 99%

Primary Biliary Cholangitis and Primary Sclerosing Cholangitis: Current Knowledge of Pathogenesis and Therapeutics

Park

Kim

et al. 2022

Biomedicines

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Cholangiopathies encompass various biliary diseases affecting the biliary epithelium, resulting in cholestasis, inflammation, fibrosis, and ultimately liver cirrhosis. Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are the most important progressive cholangiopathies in adults. Much research has broadened the scope of disease biology to genetic risk, epigenetic changes, dysregulated mucosal immunity, altered biliary epithelial cell function, and dysbiosis, all of which interact and arise in the context of ill-defined environmental triggers. An in-depth understanding of the molecular pathogenesis of these cholestatic diseases will help clinicians better prevent and treat diseases. In this review, we focus on the main underlying mechanisms of disease initiation and progression, and novel targeted therapeutics beyond currently approved treatments.

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Section: Primary Biliary Cholangitismentioning

confidence: 99%

Primary Biliary Cholangitis and Primary Sclerosing Cholangitis: Current Knowledge of Pathogenesis and Therapeutics

Park

Kim

et al. 2022

Biomedicines

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“…PBC was diagnosed based on the presence of at least two of the following criteria [ 3 ] : (1) biochemical evidence of cholestasis based on elevated ALP levels, (2) presence of AMA or AMA‐M2, and (3) diagnostic or compatible liver biopsy. Development of PBC in AMA‐positive patients was ascertained by persistent elevation of ALP and/or gamma‐glutamyl transpeptidase (GGT) or liver biopsy.…”

Section: Methodsmentioning

confidence: 99%

“…[ 1 , 2 ] Therefore, AMA has become a routine test in the work‐up of cholestatic liver diseases. [ 3 , 4 ] Of note is that most of the studies have been conducted in the setting of patients with clinical and biochemical signs of intrahepatic cholestasis, whereas the diagnostic performance of AMA in the settings of normal liver tests or non‐cholestatic profiles is less well elucidated.…”

Section: Introductionmentioning

confidence: 99%

The future risk of primary biliary cholangitis (PBC) is low among patients with incidental anti‐mitochondrial antibodies but without baseline PBC

Duan

Chen

et al. 2022

Hepatol Commun

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Anti‐mitochondrial autoantibodies (AMAs) are highly specific for the diagnosis of primary biliary cholangitis (PBC) but are also occasionally found in other diseases. In the present study, we evaluated the incidence of and predictors for PBC development in AMA‐positive patients with other liver or non‐liver diseases at baseline. In this retrospective study, we screened patients who tested positive for AMA and/or anti‐mitochondrial M2 antibody (AMA‐M2) at Beijing Friendship Hospital, Capital Medical University, from October 2005 to January 2017. They were categorized by their diagnosis at the baseline as patients with PBC or non‐PBC cases. We followed up on the non‐PBC cases through telephone interviews and reviewing of medical records to obtain laboratory results and clinical outcomes. In total, 139 patients were AMA‐positive but did not fulfill the diagnostic criteria of PBC at baseline, including 51 patients with non‐PBC liver diseases and 88 cases with non‐liver diseases. The titers of AMA‐M2, alkaline phosphatase, gamma‐glutamyl transpeptidase, and immunoglobulin M were significantly higher in patients with PBC compared to those with non‐PBC liver diseases and non‐liver diseases. After a median follow‐up of 4.6 (interquartile range: 2.4–7.6) years, 4.3% (6 of 139) developed PBC, with an accumulative 5‐year incidence rate of 4.2%. None of the patients with non‐PBC liver diseases developed PBC, whereas the 5‐year incidence rate of PBC was 7.8% among 88 patients with non‐liver diseases. Lower alanine aminotransferase and higher immunoglobulin M were independent predictors for developing PBC. Conclusion: Our results suggest a low risk of developing PBC over time in AMA‐positive patients with other liver and non‐liver diseases.

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“…UDCA still occupies a dominant position in the treatment of PBC, with its incomparable safety and effectiveness, as confirmed by several clinical trials. Fibrates are currently included in the clinical guidelines for add-on therapy ( 189 ). It is not known whether PPAR agonists will be used as monotherapy in the future or in combination with UDCA in patients with PBC, regardless of adequate response to UDCA.…”

Section: Summary and Prospectsmentioning

confidence: 99%

Peroxisome Proliferator-Activated Receptors Regulate Hepatic Immunity and Assist in the Treatment of Primary Biliary Cholangitis

et al. 2022

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Fibrates, which are agonists of peroxisome proliferator-activated receptor alpha, have received increasing attention in the treatment of primary biliary cholangitis. Reduced alkaline phosphatase levels and improved clinical outcomes were observed in patients with primary biliary cholangitis with an inadequate response to ursodeoxycholic acid (UDCA) monotherapy4 when treated with bezafibrate or fenofibrate combined with UDCA. In contrast to obeticholic acid, which exacerbates pruritus in patients, fibrates have been shown to relieve pruritus. Clinical trial outcomes show potential for the treatment of primary biliary cholangitis by targeting peroxisome proliferator-activated receptors. It is currently agreed that primary biliary cholangitis is an autoimmune-mediated cholestatic liver disease, and peroxisome proliferator-activated receptor is a nuclear receptor that regulates the functions of multiple immune cells, thus playing an important role in regulating innate and adaptive immunity. Therefore, this review focuses on the immune disorder of primary biliary cholangitis and summarizes the regulation of hepatic immunity when peroxisome proliferator-activated receptors are targeted for treating primary biliary cholangitis.

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

Abstract: Consent to participate Not applicable.Consent for publication Written informed consent for publication was obtained from all participants.

Cited by 61 publications

References 239 publications

Primary Biliary Cholangitis and Primary Sclerosing Cholangitis: Current Knowledge of Pathogenesis and Therapeutics

Primary Biliary Cholangitis and Primary Sclerosing Cholangitis: Current Knowledge of Pathogenesis and Therapeutics

The future risk of primary biliary cholangitis (PBC) is low among patients with incidental anti‐mitochondrial antibodies but without baseline PBC

Peroxisome Proliferator-Activated Receptors Regulate Hepatic Immunity and Assist in the Treatment of Primary Biliary Cholangitis

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