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World Journal of Clinical Cases 2024年第6期12卷 1174-1181页

作者： Yu-Jie Qin Ting Gao Xing-Nian Zhou Ming-Liang Cheng Hong Li Clinical Medicine School of Guizhou Medical University Guiyang 550025Guizhou ProvinceChina Department of Pathology The Affiliated Hospital of Guizhou Medical UniversityGuiyang 550025Guizhou ProvinceChina Department of Infectious Diseases Guizhou Medical UniversityGuiyang 550025Guizhou ProvinceChina Department of Infectious Diseases Guizhou Provincial People's HospitalGuiyang 550025Guizhou ProvinceChina

BACKGROUND Autoimmune hepatitis(AIH)and primary biliary cholangitis(PBC)are two common clinical autoimmune liver diseases,and some patients have both diseases;this feature is called AIH-PBC overlap syndrome.Autoimmune thyroid disease(AITD)is the most frequently overlapping extrahepatic autoimmune disease.Immunoglobulin(IgG)4-related disease is an autoimmune disease recognized in recent years,characterized by elevated serum IgG4 levels and infiltration of IgG4-positive plasma cells in tissues.CASE SUMMARY A 68-year-old female patient was admitted with a history of right upper quadrant pain,anorexia,and jaundice on physical examination.Laboratory examination revealed elevated liver enzymes,multiple positive autoantibodies associated with liver and thyroid disease,and imaging and biopsy suggestive of pancreatitis,hepatitis,and PBC.A diagnosis was made of a rare and complex overlap syndrome of AIH,PBC,AITD,and IgG4-related disease.Laboratory features improved on treatment with ursodeoxycholic acid,methylprednisolone,and azathioprine.CONCLUSION This case highlights the importance of screening patients with autoimmune diseases for related conditions.

关键词： Overlap syndrome Autoimmune hepatitis primary biliary cholangitis primary sclerosing cholangitis Autoimmune thyroid disease Case report

Study on the effect and mechanism of Swertia mussotii Franch. in the treatment of primary biliary cholangitis based on bioinformatics and in vitro experiments

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Traditional Medicine Research 2024年第3期9卷 32-42页

作者： Xing-Fang Zhang Meng-Meng Yang Yi-Chen Guo Meng-Yuan Wang Hong-Xia Yang Ming Zhang Cen Li Li-Xin Wei Hong-Tao Bi Qinghai Provincial Key Laboratory of Tibetan Medicine Pharmacology and Safety Evaluation Northwest Institute of Plateau BiologyChinese Academy of ScienceXining 810008China Medical College Qinghai UniversityXining 810001China College of Eco-Environmental Engineering Qinghai UniversityXining 810016China CAS Key Laboratory of Tibetan Medicine Research Northwest Institute of Plateau BiologyChinese Academy of SciencesXining 810001China

Background:primary biliary cholangitis(PBC)is a chronic biliary autoimmune liver disease characterized by intrahepatic cholestasis.Swertia mussotii Franch.(SMF)is a Tibetan medicine with hepatoprotective and anti-inflammatory activities.In this study,the therapeutic effect and potential mechanisms of SMF on PBC were investigated by bioinformatics analysis and in vitro experimental validation,with the aim of promoting the progress of SMF and PBC research.Methods:We first explored the therapeutic effects and key targets of SMF on PBC using a network pharmacology approach,further screened the core targets using the GSE79850 dataset,and finally validated the results using molecular docking techniques and in vitro experiments.Results:By bioinformatics analysis,we identified core targets of SMF for PBC treatment(STAT3,JAK2,TNF-α,and IL-1β)and important signaling pathways:JAK-STAT,TNF,and PI3K-AKT.The molecular docking results showed that the significant components of SMF had good binding properties to the core targets.In vitro experiments showed that SMF extracts improved the extent of epithelial-mesenchymal transition in human intrahepatic biliary epithelial cells and had a significant reversal effect on epithelial-mesenchymal transition process markers and potential targets in PBC.Conclusion:SMF may exert its therapeutic effects on PBC by acting on important targets such as STAT3,JAK2,TNF-α,IL-1β,Vimentin,and E-cadherin and the pathways in which they are involved.

关键词： Swertia mussotii Franch. primary biliary cholangitis bioinformatics in vitro experiments

primary biliary cholangitis in pregnancy:A systematic review with meta-analysis

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Hepatobiliary & Pancreatic Diseases International 2022年第3期21卷 218-225页

作者： Hydar El Jamaly Guy D Eslick Martin Weltman Department of Gastroenterology and Hepatology Nepean HospitalPenrithNew South WalesAustralia Nepean Clinical School The University of SydneyPenrithNew South WalesAustralia NHMRC Centre for Research Excellence in Digestive Health Hunter Medical Research InstituteThe University of NewcastleNewcastleNew South WalesAustralia

Background: The outcomes and disease associations in pregnant women with primary biliary cholangitis(PBC) have not been largely explored. This study aimed to determine the level of evidence associated with maternal and fetal outcomes and other disease associations in female patients with PBC. Data sources:: A comprehensive literature search was conducted. Maternal and fetal outcomes were obtained from patients with a previous, current or subsequent diagnosis of PBC. A random-effects model was employed, using odds ratios(ORs) with 95% confidence intervals(CIs). Results: Eleven studies, with 2179 female PBC patients were included. Pregnant women with PBC were significantly more likely to have a miscarriage(OR = 1.27, 95% CI: 1.02-1.58;P = 0.03), and a history of abortion(OR = 1.50, 95% CI: 1.09-2.07;P = 0.01), with absent heterogeneity( I 2 = 0%). PBC pregnant women were significantly more likely to deliver via vaginal birth(OR = 1.69, 95% CI: 1.33-2.14;P < 0.001) with low level heterogeneity( I^(2) < 0.001%). Patients had a statistically significant increased likelihood of lifetime smoking(OR = 1.95, 95% CI: 1.17-3.23;P = 0.01). Egger’s regression revealed no evidence of publication bias. Conclusions: This meta-analysis provides pooled evidence that a PBC pregnancy is associated with fetal morbidity and maternal lifestyle associations that may influence pregnancy outcomes. More studies are needed to establish disease associations that may directly affect pregnancy outcomes. These data are essential for clinicians managing these patients before, during or after pregnancy.

关键词： Pregnancy Fetal Maternal primary biliary cholangitis Miscarriage

Concomitant extrahepatic autoimmune diseases do not compromise the long-term outcomes of primary biliary cholangitis

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Hepatobiliary & Pancreatic Diseases International 2022年第6期21卷 577-582页

作者： Sha Chen Meng-Qi Li Wei-Jia Duan Bu-Er Li Shu-Xiang Li Ting-Ting Lv Lin Ma Ji-Dong Jia Liver Research Center Beijing Friendship HospitalCapital Medical UniversityBeijing Key Laboratory of Translational Medicine on Liver CirrhosisNational Clinical Research Center for Digestive Diseases95 Yong’an RoadBeijing 100050China

Background:primary biliary cholangitis(PBC)patients often have concomitant extrahepatic autoimmune(EHA)diseases including Sjögren’s syndrome(SS),systemic sclerosis(SSc),rheumatoid arthritis(RA),and autoimmune thyroid disease.The present study aimed to describe the prevalence of EHA diseases in PBC and explore the impact of EHA diseases on the long-term outcomes of PBC in Chinese patients.Methods:Medical records of PBC patients diagnosed in our institute were retrospectively reviewed.Pa-tients were followed up by a standardized telephone interview.The endpoints were defined as liver-related death and/or liver transplantation.Results:Totally 247 of the 985(25.1%)PBC patients enrolled in the study had at least one concomi-tant EHA disease.Sjögren’s syndrome(n=140,14.2%)was the most frequent one,followed by rheuma-toid arthritis(RA)(n=56,5.7%)and Hashimoto’s thyroiditis(n=45,4.6%).Patients with EHA dis-eases were more common in females(P<0.001)and in those with a family history of autoimmune disease(P=0.017).Overall,no differences were found between PBC patients with and without EHA dis-eases in terms of biochemical response rates to ursodeoxycholic acid,the incidence of hepatic events,or transplant-free survival.RA and EHA≥2 were protective factors for hepatic events in univariate Cox analysis,but the results became insignificant in multivariate analysis.Conclusions:Concomitant EHA diseases were common in PBC patients but did not compromise the long-term outcomes of PBC.

关键词： primary biliary cholangitis Extrahepatic autoimmune diseases Sjögren’s syndrome Rheumatoid arthritis Prognosis

Mapping of de novo mutations in primary biliary cholangitis to a disease-specific co-expression network underlying homeostasis and metabolism

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Journal of Genetics and Genomics 2022年第2期49卷 145-154页

作者： Lu Wang Jinchen Li Chan Wang Ruqi Tang Jialong Liang Yuhua Gong Yaping Dai Ningling Ding Jian Wu Na Dai Lei Liu Yi Zhao Youlin Shao Weifeng Zhao Peng Jiang Xingjuan Shi Weichang Chen Ye Tian Xiangdong Liu Xiong Ma Zhongsheng Sun Key Laboratory of Developmental Genes and Human Diseases Institute of Life SciencesSoutheast University2 Sipailou RoadNanjingJiangsu 210096China Beijing Institutes of Life Science Chinese Academy of SciencesNo.1 West Beichen RoadChaoyang DistrictBeijing 100101China Department of Gastroenterology and Hepatology Shanghai Institute of Digestive DiseasesShanghai Jiao Tong University School of Medicine Afﬁliated Renji Hospital145 Shandong Middle RoadShanghai 200001China Department of Laboratory Medicine The Third People’s Hospital of Zhenjiang300 DaijiamenZhenjiangJiangsu 212021China Department of Laboratory Medicine The Fifth People’s Hospital of Wuxi1215 Guangrui RoadWuxiJiangsu 214000China Department of Hepatology The Fifth People’s Hospital of SuzhouSoochow University10 Guangqian RoadSuzhouJiangsu 215131China Department of Rheumatology First Afﬁliated Hospital of Soochow University188 Shizi RoadSuzhouJiangsu 215006China Department of Gastroenterology Jiangsu University Afﬁliated Kunshan Hospital91 Qianjin West RoadKunshanJiangsu 215300China Department of Gastroenterology Yixing People’s Hospital75 Tongzhenguan RoadYixinJiangsu 214200China Department of Gastrointestinal Endoscopy Eastern Hepatobiliary Surgery Hospital700 Moyu North RoadShanghai 201800China Department of Hepatology The Third People’s Hospital of Changzhou300 Lanling North RoadChangzhouJiangsu 213001China Department of Infectious Diseases First Afﬁliated Hospital of Soochow University188 Shizi RoadSuzhouJiangsu 215006China Department of Gastroenterology First Afﬁliated Hospital of Soochow University188 Shizi RoadSuzhouJiangsu 215006China Department of Radiology Second Afﬁliated Hospital of Soochow University1055 Sanxiang RoadSuzhouJiangsu 215004China Institute of Genomic Medicine Wenzhou Medical UniversityUniversity TownChashanWenzhouZhejiang 325035China

primary biliary cholangitis(PBC) is an autoimmune disease involving dysregulation of a broad array of homeostatic and metabolic processes. Although considerable single-nucleotide polymorphisms have been unveiled, a large fraction of risk factors remains enigmatic. Candidate genes with rare mutations that tend to confer more deleterious effects need to be identified. To help pinpoint cellular and developmental mechanisms beyond common noncoding variants, we integrate whole exome sequencing with integrative network analysis to investigate genes harboring de novo mutations. Prominent convergence has been revealed on a network of disease-specific co-expression comprised of 55 genes associated with homeostasis and metabolism. The transcription factor gene MEF2 D and the DNA repair gene PARP2 are highlighted as hub genes and identified to be up-and down-regulated, respectively, in peripheral blood data set. Enrichment analysis demonstrates that altered expression of MEF2 D and PARP2 may trigger a series of molecular and cellular processes with pivotal roles in PBC pathophysiology. Our study identifies genes with de novo mutations in PBC and suggests that a subset of genes in homeostasis and metabolism tend to act in synergy through converging on co-expression network, providing novel insights into the etiology of PBC and expanding the pool of molecular candidates for discovering clinically actionable biomarkers.

关键词： primary biliary cholangitis de novo mutations Whole exome sequencing Transcriptional networks Co-expression

Clinical utility of two-dimensional shear-wave elastography in monitoring disease course in autoimmune hepatitis-primary biliary cholangitis overlap syndrome

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World Journal of Gastroenterology 2022年第18期28卷 2021-2033页

作者： Yu-Ling Yan Xian Xing Yi Wang Xiao-Ze Wang Zhi Wang Li Yang Department of Gastroenterology and Hepatology West China HospitalSichuan UniversityChengdu 610041Sichuan ProvinceChina Sichuan University University of Oxford Huaxi Joint for Gastrointestinal Cancer CentreChengdu 610207Sichuan ProvinceChina

BACKGROUND Autoimmune hepatitis-primary biliary cholangitis(AIH-PBC)overlap syndrome has a worse prognosis than AIH or PBC alone.Therefore,accurately staging liver fibrosis and dynamically monitoring disease progression are essential.AIM To investigate the performance of two-dimensional shear-wave elastography(2DSWE)for noninvasively staging liver fibrosis and assessing the clinical utility of repeated 2D-SWE for monitoring treatment response in AIH-PBC overlap syndrome.METHODS A total of 148 patients diagnosed with AIH-PBC overlap syndrome were retrospectively enrolled.Among them,82 patients had a 2D-SWE follow-up time of more than 1 year.The Scheuer scoring system was used to evaluate stages of hepatic inflammation and liver fibrosis.The performance of 2D-SWE for staging liver fibrosis was evaluated with the liver biopsy.Changes in liver stiffness(LS)measured by 2D-SWE in patients with or without complete biochemical remission were evaluated.RESULTS LS value was strongly correlated with liver fibrosis stage(Spearman r=0.84,P<0.0001).The areas under the receiver operating characteristic curves of LS for diagnosing significant fibrosis(≥S2),severe fibrosis(≥S3),and cirrhosis(S4)were 0.91,0.97,and 0.96,respectively.Patients with complete biochemical remission had a considerable decrease in LS values(P<0.0001).More importantly,the declined LS in patients with S0-S2 was significantly lower than that in patients with S3-S4(P=0.0002).In contrast,patients who failed to achieve biochemical remission had a slight but not significant decrease in LS(P=0.37).CONCLUSION LS measured by 2D-SWE is an accurate and reliable method in assessing liver fibrosis,especially for diagnosing severe fibrosis(≥3)and monitoring treatment response in patients with AIH-PBC overlap syndrome.

关键词： Liver fibrosis Liver biopsy Diagnostic tests Autoimmune hepatitis primary biliary cholangitis Inflammation

Serum metabolic profiling of targeted bile acids reveals potentially novel biomarkers for primary biliary cholangitis and autoimmune hepatitis

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World Journal of Gastroenterology 2022年第39期28卷 5764-5783页

作者： Zhen-Hua Ma Xiao-Mei Wang Rui-Hong Wu Da-Lin Hao Li-Chao Sun Pan Li Jun-Qi Niu Department of Infection and Hepatology The Affiliated Hospital of Beihua UniversityJilin 132011Jilin ProvinceChina Department of Hepatology The First Hospital of Jilin UniversityChangchun 130061Jilin ProvinceChina Department of Pathology The Affiliated Hospital of Beihua UniversityJilin 132011Jilin ProvinceChina

BACKGROUND primary biliary cholangitis(PBC)and autoimmune hepatitis(AIH)are two unexplained immune diseases.The golden standard for diagnosis of these diseases requires a liver biopsy.Liver biopsy is not widely accepted by patients because of its invasive nature,and atypical liver histology can confuse diagnosis.In view of the lack of effective diagnostic markers for PBC and AIH,combined with the increasingly mature metabolomics technologies,including full-contour metabolomics and target.AIM To determine non-invasive,reliable,and sensitive biochemical markers for the differential diagnosis of PBC and AIH.METHODS Serum samples from 54 patients with PBC,26 patients with AIH and 30 healthy controls were analyzed by Ultra-high performance liquid chromatographytandem mass spectrometry serum metabolomics.The metabolites and metabolic pathways were identified,and the metabolic changes,metabolic pathways and inter-group differences between PBC and AIH were analyzed.Fifteen kinds of target metabolites of bile acids(BAs)were quantitatively analyzed by SRM,and the differential metabolites related to the diagnosis of PBC were screened by receiver operating characteristic curve analysis.RESULTS We found the changes in the levels of amino acids,BAs,organic acids,phospholipids,choline,sugar,and sugar alcohols in patients with PBC and AIH.Furthermore,the SRM assay of BAs revealed the increased levels of chenodeoxycholic acid,lithocholic acid(LCA),taurolithocholic acid(TLCA),and LCA+TLCA in the PBC group compared with those in the AIH group.The levels of BAs may be used as biomarkers to differentiate PBC from AIH diseases.The levels of glycochenodeoxycholic acid,glycochenodeoxycholic sulfate,and taurodeoxycholic acid were gradually elevated with the increase of Child-Pugh class,which was correlated with the severity of disease.CONCLUSION The results demonstrated that the levels of BAs could serve as potential biomarkers for the early diagnosis and assessment of the severity of PBC and AIH.

关键词： primary biliary cholangitis Autoimmune hepatitis Biomarkers Serum metabolic profiling Bile acids Ultra-performance liquid chromatography-quadrupole time-of-flight mass spectrometry

Zebrafish ppp1r21 mutant as a model for the study of primary biliary cholangitis

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Journal of Genetics and Genomics 2023年第12期50卷 1004-1013页

作者： Chaoying Wu Wenfeng Zhang Yiyu Luo Chaoqing Cheng Xinjuan Wang Yan Jiang Shuang Li Lingfei Luo Yun Yang Institute of Developmental Biology and Regenerative Medicine Southwest UniversityBeibeiChongqing 400715China

primary biliary cholangitis (PBC) is an autoimmune cholestatic liver disease that progresses to fibrosis and cirrhosis, resulting from the gradual destruction of intrahepatic bile ducts. Exploring genetic variants associated with PBC is essential to understand the pathogenesis of PBC. Here we identify a zebrafish balloon dog (blg) mutant with intrahepatic bile duct branching defects, exhibiting several key pathological PBC-like features, including immunodominant autoantigen PDC-E2 production, cholangiocyte apoptosis, immune cell infiltration, inflammatory activation, and liver fibrosis. blg encodes the protein phosphatase 1 regulatory subunit 21 (Ppp1r21), which is enriched in the liver and its peripheral tissues and plays a vital role in the early intrahepatic bile duct formation stage. Further studies show an excessive activation of the PI3K/AKT/mTOR pathway in the hepatic tissues in the mutant, while treatment with the pathway inhibitor LY294002 and rapamycin partially rescues intrahepatic bile duct branching defects and alleviates the PBC-like symptoms. These findings implicate the potential role of the Ppp1r21-mediated PI3K/AKT/mTOR pathway in the pathophysiology of PBC.

关键词： Zebrafish mutant ppp1r21 primary biliary cholangitis PDC-E2 PI3K/AKT/mTOR pathway

Guidelines on the Diagnosis and Management of primary biliary cholangitis (2021)

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Journal of Clinical and Translational Hepatology 2023年第3期11卷 736-746页

作者： Hong You Weijia Duan Shuxiang Li Tingting Lv Sha Chen Lungen Lu Xiong Ma Ying Han Yuemin Nan Xiaoyuan Xu Zhongping Duan Lai Wei Jidong Jia Hui Zhuang Chinese Society of Hepatology,Chinese Medical Association Liver Research Center Beijing Friendship HospitalCapital Medical University&National Clinical Research Center for Di-gestive DiseasesBeijingChina Department of Gastroenterology First People’s HospitalShanghai Jiao Tong University School of MedicineShanghaiChina Department of Gastroenterology and Hepatology Renji HospitalShanghai Jiao Tong UniversityShanghaiChina Department of Gastroenterology Xijing HospitalFourth Military Medical UniversityXi’anShaanxiChina Department of Traditional and Western Medical Hepatology The Third Hospital of Hebei Medical UniversityShijiazhuangHebeiChina Department of Infectious Diseases and Center for Liver Diseases Peking University First Hos-pitalBeijingChina Artificial Liver Center Beijing You-An HospitalCapital Medical UniversityBeijingChina Hepatobil-iary Pancreatic Center Tsinghua Changgung HospitalTsinghua UniversityBeijingChina Department of Microbiology and Infectious Disease Center School of Basic Medical SciencesPeking University Health Science CenterBeijingChina 不详

In 2015, the Chinese Society of Hepatology and the Chinese Society of Gastroenterology published a consensus on pri-mary biliary cholangitis (PBC). In the past years, numerous clinical studies have been published in the field of PBC. To guide the clinical diagnosis and management of PBC patients, the Chinese Society of Hepatology invited a panel of experts to assess the new clinical evidence and formulate the current guidelines.

关键词： primary biliary cholangitis Guidance Diagnosis Management Chinese

Role of biochemical markers and autoantibodies in diagnosis of early-stage primary biliary cholangitis

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世界胃肠病学杂志:英文版 2023年第34期29卷 5075-5081页

作者： Yu-Jin Zhu Jing Li Yong-Gang Liu Yong Jiang Xiao-Jing Cheng Xu Han Chun-Yan Wang Jia Li Graduate School Tianjin Medical UniversityTianjin 300041China Department of Pathology Clinical School of the Second People's HospitalTianjin 300110China Department of Gastroenterology The Second Hospital of Tianjin Medical UniversityTianjin 300211China Department of Gastroenterology Clinical School of the Second People's HospitalTianjin 300110China

BACKGROUND primary biliary cholangitis(PBC)is a chronic progressive autoimmune cholestatic disease.The main target organ of PBC is the liver,and nonsuppurative inflammation of the small intrahepatic bile ducts may eventually develop into cirrhosis or liver fibrosis.AIM To explore the clinical characteristics of early-stage PBC,identify PBC in the early clinical stage,and promptly treat and monitor PBC.METHODS The data of 82 patients with PBC confirmed by pathology at Tianjin Second People’s Hospital from January 2013 to November 2021 were collected,and the patients were divided into stage I,stage II,stage III,and stage IV according to the pathological stage.The general data,serum biochemistry,immunoglobulins,and autoimmune antibodies of patients in each stage were retrospectively analyzed.RESULTS In early-stage(stages I+II)PBC patients,50.0%of patients had normal alanine aminotransferase(ALT)levels,and 37.5%had normal aspartate aminotransferase(AST)levels.For the remaining patients,the ALT and AST levels were mildly elevated;all of these patients had levels of5 times the upper limit of normal value.Moreover,γ-glutamyl transferase(GGT)was more robustly elevated,as 29.2%of patients had GGT levels of>10 times the upper limit of normal value.The ALP values among the three groups were significantly different(P<0.05).In early stage,the jaundice index did not increase significantly,but it gradually increased with disease progression.However,the above indicators were significantly different(P<0.05)between the early-stage group and the stage IV group.With the progression of the disease,the levels of albumin and albumin/globulin ratio tended to decrease,and the difference among the three groups was statistically si

关键词： primary biliary cholangitis Early stage Biochemical makers Autoantibodies Pathology