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Immunoglobulin G4-related sclerosing cholangitis:A case report 认领 引用
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作者 Yu Xia Jun-Da Zhu +4 位作者 Tong Su Lei Shi Shu-Lei Zhao Hong-Wei Xu Chang-Qin Xu 《World Journal of Gastrointestinal Endoscopy》 2026年第4期97-106,共10页
BACKGROUND Immunoglobulin G4-related sclerosing cholangitis(IgG4-SC)is a rare autoimmune disorder commonly associated with autoimmune pancreatitis.Because its clinical manifestations are nonspecific,the condition is f... BACKGROUND Immunoglobulin G4-related sclerosing cholangitis(IgG4-SC)is a rare autoimmune disorder commonly associated with autoimmune pancreatitis.Because its clinical manifestations are nonspecific,the condition is frequently misdiagnosed as primary sclerosing cholangitis or cholangiocarcinoma.Early recognition and timely management are essential for improving patient outcomes.CASE SUMMARY An 88-year-old male presented with jaundice,epigastric pain,and biochemical evidence of cholestasis.Laboratory tests revealed markedly elevated serum IgG4(2460 mg/dL),and imaging studies demonstrated bile duct wall thickening with multiple strictures.Histopathological examination showed minimal fibrosis accompanied by chronic inflammatory infiltration.After excluding malignancy,a diagnosis of isolated IgG4-SC was established.Steroid therapy with prednisone(40 mg/day)led to significant improvement in symptoms and biochemical markers.Follow-up imaging confirmed regression of the biliary strictures.CONCLUSION Isolated IgG4-SC mimics primary sclerosing cholangitis or cholangiocarcinoma,making early,accurate differentiation crucial to guide therapy and avoid unnecessary surgery. 展开更多
关键词 Immunoglobulin G4-related sclerosing cholangitis Isolated Immunoglobulin G4-related sclerosing cholangitis Autoimmune pancreatitis Primary sclerosing cholangitis Case report
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Fatigue and circadian rhythm in non-cirrhotic primary biliary cholangitis:An exploratory comparison with primary sclerosing cholangitis and healthy controls 认领 引用
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作者 Armando Curto Michele Tanturli +8 位作者 Rocco Gabriele Iamello Pietro Rossi Giulia Mengozzi Leonardo Dei Tommaso Mello Tommaso Innocenti Gabriele Dragoni Andrea Galli Erica Nicola Lynch 《World Journal of Hepatology》 2026年第2期224-234,共11页
BACKGROUND Fatigue is common and debilitating in primary biliary cholangitis(PBC)without clear relation to disease stage;mechanisms remain unclear.Circadian disruption is reported in end-stage liver disease,but eviden... BACKGROUND Fatigue is common and debilitating in primary biliary cholangitis(PBC)without clear relation to disease stage;mechanisms remain unclear.Circadian disruption is reported in end-stage liver disease,but evidence in non-cirrhotic PBC,and links to fatigue,is scarce.AIM To investigate the severity and phenotype of fatigue,daytime sleepiness,and chronotype in non-cirrhotic PBC,and compare findings with matched healthy controls(HC)and non-cirrhotic primary sclerosing cholangitis(PSC).METHODS Participants completed the Fatigue Impact Scale,Epworth Sleepiness Scale,and Morningness-Eveningness Questionnaire Self-Assessment.Demographics,sleep habits,employment,and fatigue subtype(mental vs muscular)were recorded.In PBC/PSC,PBC-40 fatigue/itch domains and disease characteristics were analyzed.Group comparisons and multivariable models evaluated associations of fatigue phenotype and chronotype with demographic/disease variables.RESULTS We enrolled 152 individuals:61 PBC,30 PSC,and 61 HC.Global fatigue scores did not differ across groups.Muscular fatigue predominated in PBC/PSC,whereas HC more often reported mental fatigue;this pattern persisted after stratifying by employment and sex.In adjusted analyses,HC had lower odds of muscular fatigue than PBC[odd ratio(OR)=0.30,95%confidence interval(CI):0.14-0.63;P=0.002];PSC did not differ from PBC(OR=1.25,95%CI:0.44-3.71;P=0.681).Older age independently increased the odds of muscular fatigue(OR=1.04 per year,95%CI:1.01-1.07;P=0.016).Daytime sleepiness was low and similar between groups.Morningness-Eveningness Questionnaire Self-Assessment scores clustered in the intermediate range;age,not disease,predicted greater morningness.CONCLUSION In non-cirrhotic PBC,fatigue shows a predominantly muscular phenotype independent of demographics/occupation and decoupled from conventional disease metrics.Chronotype and daytime alertness appear largely preserved,with age driving modest morningness.Findings support integrating fatigue phenotyping into assessment,prioritizing muscle-targeted interventions,and using carefully selected controls and objective endpoints in future studies. 展开更多
关键词 Chronotype Daytime sleepiness Cholestatic disorders Mental Muscular Primary biliary cholangitis Primary sclerosing cholangitis
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Patients with primary sclerosing cholangitis with and without endstage liver disease have similar outcomes after liver transplantation 认领 引用
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作者 Mojmir Hlavaty Jan Brezina +6 位作者 Pavel Wohl Istvan Modos Lukas Bajer Ondrej Fabian Andrea Vajsova TomasHucl Pavel Drastich 《World Journal of Hepatology》 2026年第3期183-193,共11页
BACKGROUND Liver transplantation(LT)is the most effective treatment for the advanced stages of primary sclerosing cholangitis(PSC).However,up to 30%of patients develop recurrence of PSC(rPSC),which negatively affects ... BACKGROUND Liver transplantation(LT)is the most effective treatment for the advanced stages of primary sclerosing cholangitis(PSC).However,up to 30%of patients develop recurrence of PSC(rPSC),which negatively affects graft and patient outcomes.Given the heterogeneous nature of PSC,patients undergo LT either due to endstage liver disease(ESLD)or to symptoms that significantly reduce quality of life(non-ESLD),such as recurrent bacterial cholangitis or refractory pruritus.However,it remains unclear whether these indicators influence post-LT outcomes.AIM To compare post-LT outcomes between PSC recipients with ESLD and non-ESLD indications,and identify rPSC and graft failure risk factors.METHODS This single-center retrospective study comprised 131 adult LT recipients for PSC(including PSC/autoimmune overlap).Patients were grouped by listing indication for comparison of ESLD vs non-ESLD.ESLD was defined as model for ESLD score≥15 and Child-Pugh score≥8,or≥1 sign of decompensated cirrhosis.Time-to-event outcomes were analyzed using Kaplan-Meier and Cox models with time-updated covariates.RESULTS No significant difference was found in the incidence of rPSC,graft survival,or overall survival between patients indicated for LT for ESLD and those with nonadvanced symptomatic disease.Cytomegalovirus infection(hazard ratio[HR]=2.16;95%confidence interval[CI]:1.05-4.46),acute cellular rejection(ACR)(HR=3.95;95%CI:1.44-10.8),and length of hospitalization after LT(HR=1.02;95%CI:1.01-1.04)were significantly associated with risk of rPSC.In addition,multiple episodes of ACR(HR=4.93;95%CI:1.22-19.9)and the length of hospitalization after LT(HR=1.04;95%CI:1.01-1.06)were significantly associated with graft failure.CONCLUSION Patients with PSC with advanced liver cirrhosis before LT did not have worse post-transplant outcomes than those without ESLD.Cytomegalovirus infection,ACR,and prolonged hospitalization after LT were associated with worse outcomes after LT in PSC. 展开更多
关键词 Primary sclerosing cholangitis Liver transplant Recurrence of primary sclerosing cholangitis Cytomegalovirus Acute cellular rejection Overlap syndrome Graft failure
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Ocular surface characteristics and dry eye symptoms in primary biliary cholangitis patients complicated with seasonal allergic conjunctivitis 认领 引用
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作者 Fang Ruan Wen-Tao Peng +3 位作者 Lian-Yong Xie Ying Han Bin Xu Wen-Jun Kong 《International Journal of Ophthalmology(English edition)》 SCIE CAS 2026年第4期681-687,共7页
AIM:To evaluate ocular surface characteristics and dry eye symptoms in primary biliary cholangitis(PBC)patients with seasonal allergic conjunctivitis(SAC).METHODS:This retrospective case-control study included 73 PBC ... AIM:To evaluate ocular surface characteristics and dry eye symptoms in primary biliary cholangitis(PBC)patients with seasonal allergic conjunctivitis(SAC).METHODS:This retrospective case-control study included 73 PBC patients:38 with SAC(PBC+SAC group,38 eyes)and 35 controls(35 eyes).All the participants underwent comprehensive ocular surface assessment and ocular surface disease index(OSDI)scoring.The severity of ocular allergy in PBC+SAC group was indicated by total ocular symptom score(TOSS),and correlation analysis was carried out between TOSS and ocular parameters.RESULTS:Age(59.60±10.69y vs 58.72±11.13y,P=0.629)and gender distribution(34 vs 32 females,P=0.713)did not differ significantly between groups.Compared to controls,the PBC+SAC group had higher dry eye prevalence(65.79%vs 28.57%,P=0.002),higher OSDI score[median:34.3(interquartile range,IQR:26.05,43.6)vs 21.2(IQR:15.15,31.2),P0.05).CONCLUSION:PBC patients with SAC have higher dry eye prevalence and more severe dry eye symptoms,mainly evaporative dry eye with obvious upper meibomian gland morphological abnormalities.Allergic symptom severity is positively correlated with dry eye discomfort,suggesting allergic inflammation may exacerbate ocular surface burden in PBC patients. 展开更多
关键词 allergic conjunctivitis dry eye primary biliary cholangitis ocular surface meibomian gland
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Human leukocyte antigen variants and clinical features of primary biliary cholangitis:Cumulative contributions 认领 引用
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作者 Abdellatif Bouayad 《World Journal of Clinical Cases》 2026年第5期52-55,共4页
This letter to the editor highlights the importance of considering potential cumulative contributions among human leukocyte antigen(HLA)alleles in shaping the clinical manifestations of primary biliary cholangitis.Com... This letter to the editor highlights the importance of considering potential cumulative contributions among human leukocyte antigen(HLA)alleles in shaping the clinical manifestations of primary biliary cholangitis.Complementing the overview by Curto et al,which focused on non-HLA candidate genes,this paper emphasizes that specific haplotypes of HLA-DRB1,HLA-DQA1,and HLA-DQB1,as well as HLA-G*01:01:01:08/UTR-1,may modulate disease heterogeneity,predisposition to primary biliary cholangitis and autoimmune hepatitis overlap syndrome,disease progression,and poorer therapeutic response.A comprehensive understanding of these HLA polymorphisms and their interactive effects is essential for improving risk stratification and guiding personalized management of this complex autoimmune liver disease. 展开更多
关键词 Human leukocyte antigen class II Human leukocyte antigen-G Primary biliary cholangitis Prognosis Primary biliary cholangitis-autoimmune hepatitis overlap syndrome Therapy response
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Dual cut-offs and beyond:Expanding the role of transient elastography in primary biliary cholangitis 认领 引用
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作者 Vasily Isakov Alexei Goncharov 《World Journal of Gastroenterology》 SCIE CAS 2026年第8期10-17,共8页
Accurate fibrosis staging is pivotal in the management of primary biliary cholangitis(PBC).Although liver biopsy is considered the diagnostic gold standard,its invasiveness limits widespread use in disease monitoring.... Accurate fibrosis staging is pivotal in the management of primary biliary cholangitis(PBC).Although liver biopsy is considered the diagnostic gold standard,its invasiveness limits widespread use in disease monitoring.Chen et al provided strong evidence validating vibration-controlled transient elastography(VCTE)as a noninvasive alternative for identifying advanced fibrosis in Chinese patients with PBC.By establishing dual cut-offs(≤10.0 kPa and>14.5 kPa)with high diagnostic performance,the authors offer a clinically applicable tool that could substantially reduce the need for biopsy.Nevertheless,challenges persist,including the management of intermediate“grey zone”results,technical variability,and the confounding effects of cholestasis and inflammation.The integration of elastography with biochemical and prognostic indices,such as the GLOBE and UK-PBC scores,is essential for individualized care.This editorial discusses the expanding role of VCTE in PBC,current limitations,and future research directions toward standardized,integrated,noninvasive fibrosis assessment,and individualized PBC care. 展开更多
关键词 Primary biliary cholangitis Vibration-controlled transient elastography Liver stiffness measurement Prognostic value Liver fibrosis staging
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Adipokine profiles reflect metabolic dysfunction but not fibrosis in patients with primary biliary cholangitis 认领 引用 被引量：1
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作者 Tomas Koky Sylvia Drazilova +7 位作者 Slavomira Komarova Marian Macej Dominika Toporcerova Martin Janicko Ivana Spakova Miroslava Rabajdova Maria Marekova Peter Jarcuska 《World Journal of Hepatology》 2026年第2期209-223,共15页
BACKGROUND Primary biliary cholangitis(PBC)is a rare,nonsuppurative cholestatic disease that affects the small intrahepatic bile ducts.If not adequately managed,it may progress to liver cirrhosis and hepatocellular ca... BACKGROUND Primary biliary cholangitis(PBC)is a rare,nonsuppurative cholestatic disease that affects the small intrahepatic bile ducts.If not adequately managed,it may progress to liver cirrhosis and hepatocellular carcinoma.Only a few studies have explored the impact of cardiometabolic risk factors on liver fibrosis progression in PBC.Relevant data on the role of adipokines in these processes are also limited.AIM To compare leptin and adiponectin levels in PBC patients stratified by the presence of metabolic dysfunction-associated steatotic liver disease(MASLD),metabolic syndrome(MetS),fibrosis,and biochemical response.METHODS We conducted a cross-sectional study involving 81 PBC patients diagnosed according to European Association for the Study of the Liver guidelines,all followed at a tertiary care center in Košice,Slovakia.Patients were included consecutively from the patient database in hepatology clinic in a prospective manner.Data on biochemical,clinical and anthropometric variables and their associations with MASLD,MetS,fibrosis,and biochemical response were evaluated using statistical methods including logistic regression and receiver operating characteristic analysis.RESULTS Patients with PBC/MASLD had significantly lower adiponectin levels(1698.24 pg/mL vs 2042.08 pg/mL,P=0.015)and higher leptin levels(1.89 ng/mL vs 0.62 ng/mL,P0.05).CONCLUSION Adipokines reflect metabolic status in PBC.The L/A ratio is promising biomarker for MASLD.No significant association between leptin and adiponectin levels and advanced fibrosis was detected within the limited sample size of this study. 展开更多
关键词 Primary biliary cholangitis Adipokines Liver fibrosis Metabolic dysfunction-associated steatotic liver disease Metabolic syndrome
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Timing of endoscopic retrograde cholangiopancreatography in acute cholangitis:A cohort study in Colombia 认领 引用
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作者 Juan Pablo García-Marmolejo Miguel Aguilar-Shotborgh +2 位作者 Cándida Diaz-Brochero Ana María Leguízamo-Naranjo Rómulo Vargas-Rubio 《Hepatobiliary & Pancreatic Diseases International》 SCIE CAS CSCD 2026年第2期225-228,共4页
To the Editor:Acute cholangitis is a potentially life-threatening infection of the biliary tract in which early diagnosis and timely intervention are essential to prevent rapid clinical deterioration.Biliary drainage ... To the Editor:Acute cholangitis is a potentially life-threatening infection of the biliary tract in which early diagnosis and timely intervention are essential to prevent rapid clinical deterioration.Biliary drainage through endoscopic retrograde cholangiopancreatography(ERCP)remains the cornerstone of therapy once source control is required.However,the optimal timing for this intervention continues to be debated,particularly in health systems with limited resources or restricted access to ERCP during nights and weekends. 展开更多
关键词 endoscopic retrograde cholangiopancreatography ercp remains biliary tract acute cholangitis source control biliary drainage resource limited settings endoscopic retrograde cholangiopancreatography timing
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Lean type 2 diabetes mellitus is an independent predictor of mortality in primary biliary cholangitis 认领 引用
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作者 Ting-Ting Yin Hui-Ying Lin +8 位作者 Min Zhou Jian-Wei Li Qiu-Lan Mo Hong-Jing Wang Jie Chen Hui-Ling Zhu Yu-Ting Li Meng-Yao Zheng Jin-Hui Yang 《World Journal of Gastroenterology》 SCIE CAS 2026年第13期27-39,共13页
BACKGROUND The impact of lean type 2 diabetes mellitus(T2DM)on the prognosis of patients with primary biliary cholangitis(PBC)remains unclear.AIM To explore the adverse prognostic impact of lean T2DM on liver-related ... BACKGROUND The impact of lean type 2 diabetes mellitus(T2DM)on the prognosis of patients with primary biliary cholangitis(PBC)remains unclear.AIM To explore the adverse prognostic impact of lean T2DM on liver-related mortality risk in patients with PBC.METHODS This retrospective cohort study of 877 PBC patients stratified by T2DM status and lean phenotype used Cox regression models to analyze liver-related outcomes.Interaction effect models investigated T2DM and lean phenotype synergy.Propensity score matching(1:1)controlled for confounders between lean T2DM and non-T2DM non-lean groups,with sensitivity analyses validating results.RESULTS The median age of patients was 57 years(50-66),with 716(81.64%)being female,and median follow-up was 45 months(25-48),during which 234 deaths(26.68%)occurred.After fully adjusting for confounding factors,lean T2DM remained an independent mortality risk factor in PBC patients[hazard ratio(HR)=1.845,95%confidence interval:1.219-2.793,P=0.004].Interaction effect analysis confirmed a significant synergistic effect of T2DM and lean phenotype on mortality risk(interaction term HR=2.546,95%confidence interval:1.342-4.831,P=0.004).Stratified analysis further substantiated that T2DM significantly increased mortality risk only in lean patients(HR=2.331,P<0.001),while lean phenotype elevated risk only among T2DM patients(HR=2.165,P=0.0078).Propensity score matching validated this association(HR=1.63,P=0.008),with sensitivity analyses confirming the robustness of the conclusions.CONCLUSION Lean T2DM is an independent risk factor for liver-related adverse outcomes in PBC patients,and the coexistence of T2DM and lean phenotype exhibits a significant synergistic effect on mortality risk. 展开更多
关键词 Diabetes mellitus Lean body weight Nutritional status Primary biliary cholangitis Prognosis
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Plasma exosomal miR-137 activates natural killer cells in primary biliary cholangitis by targeting NFATC1 认领 引用
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作者 Yi-Fei Yang Qi-Xuan Liu +7 位作者 Wen-Hao Su Bo Zang Bing-Qian Liu Chen-Yang Zhao Yi-Bing Han Ling-Wei Liu Patrick S C Leung Bin Liu 《World Journal of Hepatology》 2026年第6期166-178,共13页
BACKGROUND Primary biliary cholangitis(PBC)is an autoimmune liver disease involving dysregulated natural killer(NK)cell activity.AIM To investigate whether microRNAs(miRNA)modulate NK cell activation and granzyme B se... BACKGROUND Primary biliary cholangitis(PBC)is an autoimmune liver disease involving dysregulated natural killer(NK)cell activity.AIM To investigate whether microRNAs(miRNA)modulate NK cell activation and granzyme B secretion in PBC via the nuclear factor of activated T cell(NFATC)pathway,focusing on the regulatory role of miR-137-3p.METHODS Peripheral blood samples from 168 patients with PBC and 74 healthy controls were analyzed.NK cells were isolated,and miRNA expression was quantified via quantitative polymerase chain reaction.Flow cytometry assessed cluster of differentiation(CD)molecule expression,whereas dual luciferase assays validated miR-137-3p binding to the NFATC1 gene.RESULTS There were significantly lower Ct values(indicating higher expression)for miR-137-3p,miR-124-3p,and miR-506-3p in NK cells from patients with PBC vs cells from healthy controls(all P<0.05).The miR-137-3p directly bound NFATC1,enhancing granzyme B secretion(348.59±7.47 pg/mL vs 373.92±15.50 pg/mL;P<0.05)and increasing the number of CD16+NK cells(73.2%vs 46.8;P<0.05)compared to controls.CONCLUSION Exosomal miR-137-3p promotes NK cell activation in PBC by targeting NFATC1,driving CD16 expression and granzyme B secretion.These miRNAs,particularly miR-137-3p,may serve as novel diagnostic biomarkers or therapeutic targets for PBC. 展开更多
关键词 Primary biliary cholangitis Exosomes MicroRNA-137 Natural killer cells Granzyme B
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Tumor necrosis factor receptor superfamily member 13B mutationassociated chronic cholangitis with cirrhosis:A case report and review of literature 认领 引用
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作者 Ying Zhou Qi-Jiao Cheng +7 位作者 Ming-Xing Liang Su-Qun Li Yu-Zhi Su Yong-Ming Cai Zong-Qin Pan Yu-Xin Xie Ying-Hua Chen Yi-Huai He 《World Journal of Hepatology》 2026年第4期238-248,共11页
BACKGROUND The transmembrane activator and calcium-modulator and cyclophilin ligand interactor,encoded by tumor necrosis factor receptor superfamily member 13B(TNFRSF13B),maintains immune homeostasis through bidirecti... BACKGROUND The transmembrane activator and calcium-modulator and cyclophilin ligand interactor,encoded by tumor necrosis factor receptor superfamily member 13B(TNFRSF13B),maintains immune homeostasis through bidirectional regulation of B-cell function.This study presents a case of a 56-year-old female with chronic cholangitis and cirrhosis associated with a heterozygous missense mutation in TNFRSF13B.CASE SUMMARY A 56-year-old female with thalassemia trait and recurrent ascending cholangitis following cholangiojejunostomy presented with cirrhosis of unclear etiology.Despite serial hospitalizations for cholestatic decompensation and comprehensive evaluations-including serological profiling with elevated antinuclear antibody titers and histopathological assessment-no conventional cirrhosis triggers were identified.The patient presented with elevated serum immunoglobulin A(IgA)and IgG,and no apparent immune dysfunction.Two magnetic resonance imaging examinations revealed absence of the left hepatic lobe and gallbladder,no dilation of in-trahepatic bile ducts,and inflammatory lesions in the hepatic hilum,cirrhosis,and splenomegaly.Pathological review of the resected left hepatic lobe demonstrated extensive plasma cell infiltration(MUM1+/IgG+)with focal iron deposits.Whole-exome sequencing uncovered a heterozygous missense variant(c.436G>C,p.Ala146Pro)in TNFRSF13B.She was diagnosed with TNFRSF13B mutation-associated chronic cholangitis and cirrhosis.Treatment with antibiotics,hepatoprotective drugs,and ursodeoxycholic acid led to symptom resolution,and she was discharged with ongoing therapy.CONCLUSION TNFRSF13B mutation may impair B-cell negative regulation,promoting cholangitis,and cirrhosis.Iron overload and bacterial infection accelerate disease progression. 展开更多
关键词 Autoimmune liver disease Chronic cholangitis TNFRSF13B gene B cell homeostasis Case report
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Abnormalities of liver sinusoidal endothelial cells in primary biliary cholangitis 认领 引用
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作者 Evangelos Thalassinos George Notas +4 位作者 Costantinos Xidakis Ioannis Drygiannakis Ourania Sfakianaki Ioannis Tsomidis Elias Kouroumalis 《World Journal of Experimental Medicine》 2026年第1期219-231,共13页
BACKGROUND Liver sinusoidal endothelial cells(LSECs)may have a critical role in the pathogenesis of primary biliary cholangitis(PBC)that has not been investigated.AIM To investigate the role of LSECs in PBC.METHODS We... BACKGROUND Liver sinusoidal endothelial cells(LSECs)may have a critical role in the pathogenesis of primary biliary cholangitis(PBC)that has not been investigated.AIM To investigate the role of LSECs in PBC.METHODS We studied the levels of soluble vascular cell adhesion molecule-1,soluble intercellular adhesion molecule-1 and soluble E-selectin and the tissue plasminogen activator(t-PA)and its inhibitor in the serum of 30 PBC patients before and 25 patients after treatment with ursodeoxycholic acid(UDCA).Moreover,immortalized endothelial cells(EA.hy926)were incubated with serum from patients with PBC,hepatitis C virus(HCV)and normal controls for up to 24 hours.The expression of endothelin(ET)1,ET2,ET3,and ET receptors A and B were also measured by quantitative polymerase chain reaction.RESULTS Vascular cell adhesion molecule and intercellular adhesion molecule were significantly increased in PBC and HCV with the highest values found in PBC patients.UDCA had no effect.Levels were significantly higher in late PBC(stages III-IV),compared with early PBC(stages I-II).t-PA was significantly increased in PBC but not in HCV.Higher values were obtained in late PBC.UDCA decreased t-PA.Plasminogen activator inhibitor-1 levels were similar in all groups.Expression of endothelin 1,endothelin 2,and endothelin 3 significantly varied at different time points.ET receptors A was decreased at 2 hours and 6 hours in PBC,and at 2 hours and 24 hours in HCV.ET receptors B was reduced at 2 hours and 24 hours in both PBC and HCV.CONCLUSION Endothelial adhesion molecules are abnormal in PBC particularly in the late fibrotic stages.ET and their receptors are reduced in LSECs after incubation with PBC and HCV sera,findings that might be related to pathogenesis. 展开更多
关键词 Primary biliary cholangitis Sinusoidal endothelial cells Endothelial markers Endothelins Endothelin receptors
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Timing of intervention:Assessing early vs late endoscopic and surgical interventions in acute cholangitis 认领 引用
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作者 Silpa Choday Jad Alsheikh Neil Vyas 《World Journal of Gastrointestinal Endoscopy》 2026年第1期74-86,共13页
BACKGROUND Acute cholangitis(AC)is characterized by infection and inflammation of the biliary tree,often resulting from acute biliary obstruction.AIM To evaluate outcomes of endoscopic retrograde cholangiopancreatogra... BACKGROUND Acute cholangitis(AC)is characterized by infection and inflammation of the biliary tree,often resulting from acute biliary obstruction.AIM To evaluate outcomes of endoscopic retrograde cholangiopancreatography(ERCP)and laparoscopic common bile duct exploration(LCBDE)in the management of AC,focusing on timing,complications,and hospital resource utilization.METHODS Between 2016 and 2021,a total of 31817 patients were included,with 30330(95.3%)undergoing ERCP and 1487(4.67%)undergoing LCBDE.RESULTS ERCP patients were older(mean age 64.5 years vs 59.7 years;P<0.001)with higher Medicare use(56.1%vs 48.1%)compared to LCBDE patients.LCBDE patients had more elective admissions(19.6%vs 11.7%;P<0.001)and were treated more often in non-teaching hospitals(P<0.001).Complication rates differed significantly:LCBDE patients had higher respiratory failure(3.34% vs 2.34%;P=0.026)and bile duct perforation(1.55%vs 0.64%;P=0.026),while ERCP patients had higher rates of pancreatitis(P<0.001)and jaundice(P=0.002).Late ERCP was associated with higher rates of septic shock(1.23%),respiratory failure(3.80%),and bile duct perforation(0.93%)compared to earlier timing.Patients undergoing late ERCP also had longer hospital stays and higher costs(P<0.001).LCBDE patients experienced significantly longer hospital stays(mean 8.92 days vs 4.89 days)and higher costs,particularly in late interventions(P<0.001).CONCLUSION ERCP remains the preferred intervention for AC,with earlier procedures resulting in better outcomes and lower resource utilization.LCBDE,while less common,is associated with longer hospital stays and higher costs,particularly when performed late.Optimizing timing for both ERCP and LCBDE is critical to improving patient outcomes and reducing healthcare expenditures. 展开更多
关键词 Acute cholangitis Endoscopic retrograde cholangiopancreatography Laparoscopic common bile duct exploration Nationwide Readmissions Database Readmissions Timing
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Hypoalbuminemia as a predictor of mortality in patients with acute cholangitis:A systematic review and meta-analysis 认领 引用
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作者 Raja Taha Yaseen Khan Salman Ahsam +6 位作者 Sanjay Kirshan Kumar Khaiber Khan Muhammad Talha Kakar Ali Hyder Wadana Malik Muhammed Mubarak Nasir H Luck 《World Journal of Gastrointestinal Pathophysiology》 2026年第1期51-59,共9页
BACKGROUND Acute cholangitis is a potentially life-threatening infection of the biliary tract and its mortality rate is between 10%-30%.Early risk stratification is essential for the best possible outcome.Serum albumi... BACKGROUND Acute cholangitis is a potentially life-threatening infection of the biliary tract and its mortality rate is between 10%-30%.Early risk stratification is essential for the best possible outcome.Serum albumin,an index of the inflammatory and nutritional state,has been associated with adverse outcome in various acute conditions.AIM To assess the value of hypoalbuminemia as a predictor of mortality in acute cholangitis.METHODS A systematic search of PubMed,Web of Science,Semantic Scholar,Cochrane Library,and Google Scholar was performed up to May 2025.Eligible studies included adults diagnosed with acute cholangitis and reported mortality outcomes stratified by serum albumin levels.Data extraction was conducted independently by two reviewers,and study quality was assessed using the Newcastle-Ottawa Scale.Pooled odds ratios(OR)with 95%CI were calculated using a random-effects model.Heterogeneity was assessed with the I2statistic,while publication bias was evaluated through funnel plot symmetry and Egger’s regression test.RESULTS Eight retrospectively assembled cohort studies enrolling a total of 2215 of patients with acute cholangitis were incorporated in this meta-analysis.Mean age of the patient was greater than 65 years.Among them,52.2%were males.Out 2215 patients,242(10.9%)died.Hypoalbuminemia was highly associated with increased mortality.Dichotomous outcome analysis demonstrated strong evidence of association between hypoalbuminemia and mortality(OR=8.02,95%CI:3.41-18.83;P≤0.001;I2=50%).Continuous outcome analysis demonstrated a 23%increased risk of mortality associated with every 1 g/dL fall in serum albumin(OR=0.77,95%CI:0.64-0.93;P=0.005;I2=66%).All the incorporated studies were of high methodological quality with minimal evidence of publication bias.CONCLUSION Hypoalbuminemia on hospital admission is a strong and independent predictor of death in patients with acute cholangitis.Admission serum albumin measurement provides a simple,cost-effective,and universally available method of early risk stratification.Prospective studies in the future need to examine whether correction of hypoalbuminemia improves clinical outcomes. 展开更多
关键词 Acute cholangitis Hypoalbuminemia Mortality Prognosis Prediction Inflammatory marker
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Metabolic links between metabolic dysfunction-associated steatotic liver disease and primary biliary cholangitis 认领 引用
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作者 Stanislav Kotlyarov 《World Journal of Hepatology》 2026年第6期1-7,共7页
Primary biliary cholangitis(PBC)and metabolic dysfunction-associated steatotic liver disease(MASLD)increasingly coexist in the same patient against the backdrop of a global epidemic of metabolic disorders.A growing bo... Primary biliary cholangitis(PBC)and metabolic dysfunction-associated steatotic liver disease(MASLD)increasingly coexist in the same patient against the backdrop of a global epidemic of metabolic disorders.A growing body of evidence points to profound pathogenetic intersections between autoimmune damage to the biliary tract and systemic metabolic dysfunction.These interactions involve key mechanisms:Immune regulation,lipid metabolism,adipokine profile,and gut microbiota function.In comorbid cases,these processes do not simply add up,but may give rise to a qualitatively distinct clinical-pathogenetic variant.A recent study conducted by Koky et al published in World Journal of Hepatology provides important information about this comorbidity,demonstrating that patients with PBC and concomitant MASLD have a specific adipokine imbalance characterized by decreased adiponectin and increased leptin levels,resulting in an elevated leptin/adiponectin ratio.This editorial summarizes current understanding of the pathogenetic links between PBC and MASLD and discusses the implications for clinical practice and future research. 展开更多
关键词 Primary biliary cholangitis Metabolic dysfunction-associated steatotic liver disease Adipokines Adiponectin Leptin Immunometabolism Gut microbiota Oxidative stress Mitochondrial dysfunction Leptin/adiponectin ratio
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Impact of chronic proliferative cholangitis on stone recurrence and biliary strictures after intrahepatic stone treatment:A meta-analysis 认领 引用
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作者 Tauqeer Muhammad Ting-Ting He +6 位作者 Zhu-Ying Lin Jia-Lin Shi Tian-Gui Zhang Yao Yang Xiao-Lei Yang Hashim Nazir Chattha Jiang Li 《World Journal of Gastrointestinal Surgery》 SCIE 2026年第3期315-326,共12页
BACKGROUND Intrahepatic bile duct stones often come back after treatment.One possible cause is chronic proliferative cholangitis(CPC).This is a long-term inflammation of the bile ducts that can cause scarring and narr... BACKGROUND Intrahepatic bile duct stones often come back after treatment.One possible cause is chronic proliferative cholangitis(CPC).This is a long-term inflammation of the bile ducts that can cause scarring and narrowing.This inflammation may remain even after stones are removed.Its effect on stone recurrence and bile duct narrowing is still unclear.We hypothesized that CPC increases the risk of stone recurrence and bile duct strictures after treatment.AIM To determine whether CPC is associated with stone recurrence and bile duct strictures after treatment.METHODS We performed a systematic review and meta-analysis following Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines.PubMed,Scopus,Web of Science,and ScienceDirect were searched for studies published from 2015 to 2025.Only observational studies in adults treated for intrahepatic bile duct stones were included.Two reviewers independently collected data.Pooled results were calculated using random-effects statistical models.RESULTS Six observational studies including 1248 patients were analyzed.Stone removal was successful in 91% of patients.Stone recurrence occurred in 21.5% of patients.Bile duct narrowing occurred in 18% of patients.Adverse events occurred in 12% of patients.Studies reporting chronic bile duct inflammation showed higher rates of stone recurrence and bile duct narrowing.Differences between studies were moderate.CONCLUSION Stone removal is effective,but stones and bile duct narrowing often return,especially with chronic bile duct inflammation.Treating inflammation may help improve long-term results. 展开更多
关键词 Hepatolithiasis Chronic proliferative cholangitis Stone recurrence Biliary stricture Intrahepatic bile duct stones Systematic review Meta-analysis
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Bile acid therapy for primary biliary cholangitis:Pathogenetic validation 认领 引用 被引量：1
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作者 Vasiliy I Reshetnyak Igor V Maev 《World Journal of Experimental Medicine》 2025年第1期53-64,共12页
Knowledge of the etiological and pathogenetic mechanisms of the development of any disease is essential for its treatment.Because the cause of primary biliary cholangitis(PBC),a chronic,slowly progressive cholestatic ... Knowledge of the etiological and pathogenetic mechanisms of the development of any disease is essential for its treatment.Because the cause of primary biliary cholangitis(PBC),a chronic,slowly progressive cholestatic liver disease,is still unknown,treatment remains symptomatic.Knowledge of the physicochemical properties of various bile acids and the adaptive responses of cholangiocytes and hepatocytes to them has provided an important basis for the development of relatively effective drugs based on hydrophilic bile acids that can potentially slow the progression of the disease.Advances in the use of hydrophilic bile acids for the treatment of PBC are also associated with the discovery of pathogenetic mechanisms of the development of cholangiocyte damage and the appearance of the first signs of this disease.For 35 years,ursodeoxycholic acid(UDCA)has been the unique drug of choice for the treatment of patients with PBC.In recent years,the list of hydrophilic bile acids used to treat cholestatic liver diseases,including PBC,has expanded.In addition to UDCA,the use of obeticholic acid,tauroursodeoxycholic acid and norursodeoxycholic acid as drugs is discussed.The pathogenetic rationale for treatment of PBC with various bile acid drugs is discussed in this review.Emphasis is made on the mechanisms explaining the beneficial therapeutic effects and potential of each of the bile acid as a drug,based on the understanding of the pathogenesis of the initial stages of PBC. 展开更多
关键词 Primary biliary cholangitis Treatment of primary biliary cholangitis with bile acids Ursodeoxycholic acid Obeticholic acid Tauroursodeoxycholic acid Norursodeoxycholic acid
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Acute cholangitis caused by Hem-o-lok clips migration after laparoscopic left hemihepatectomy 认领 引用
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作者 Guo-Mei Ge Wei-Le Jia +4 位作者 Chen-Jun He Zhou Shao Feng Gao Jun-Jun Jia Min Zhang 《Hepatobiliary & Pancreatic Diseases International》 SCIE CAS CSCD 2025年第5期566-569,共4页
To the Editor:Acute cholangitis,also known as ascending cholangitis,is a po-tentially life-threatening condition resulting from the infection and obstruction of the biliary tract[1].Choledocholithiasis,which in-volves... To the Editor:Acute cholangitis,also known as ascending cholangitis,is a po-tentially life-threatening condition resulting from the infection and obstruction of the biliary tract[1].Choledocholithiasis,which in-volves stones in the common bile duct,is the most frequent cause of such infections,leading to partial or complete blockage of the biliary system[2].During acute episodes,patients may present with abdominal pain,high fever,and jaundice,known as Charcot’s triad,and in severe cases,patients may also exhibit signs of shock and depression[3].An episode of cholangitis can be fatal,and if the underlying obstruction is not addressed,the condition may recur,potentially leading to hepatic abscesses or biliary cirrhosis.Other etiologies of cholangitis include benign biliary strictures,bil-iary malignancies,congenital factors,postoperative complications,inflammatory conditions,and rare causes such as pancreatitis,par-asitic invasion of the biliary tract,external compression,thrombo-sis,and iatrogenic factors.Acute cholecystitis due to the migration of Hem-o-lok clips postoperation is rare.Herein we reported a case of acute cholangitis caused by the migration of surgical Hem-o-lok clips into the bile duct six years after laparoscopic left hemihepa-tectomy. 展开更多
关键词 common bile ductis ascending cholangitisis Acute cholangitis obstruction biliary tract choledocholithiasiswhich Biliary tract obstruction Laparoscopic left hemihepatectomy biliary system Hem o lok clips migration
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MicroRNA-126-3p as a predictive biomarker for patients with primary biliary cholangitis refractory to ursodeoxycholic acid 认领 引用 被引量：1
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作者 Shi-Da Pan Chu-Yue Xiong +13 位作者 Ying-Juan Shen Jia-He Tian Yi-Lin Wang Jia-Ning Wang Si-Yu Wang Feng-Yi Li Li-Feng Wang Qin Qiu Luo Yang Xiao-Meng Liu Jun-Qing Luan Zheng-Sheng Zou Fu-Sheng Wang Fan-Ping Meng 《World Journal of Gastroenterology》 SCIE CAS 2025年第31期75-91,共17页
BACKGROUND Ursodeoxycholic acid(UDCA)is the first-line therapeutic agent for primary biliary cholangitis(PBC).However,a subset of patients exhibit a suboptimal response to UDCA,and reliable predictive biomarkers remai... BACKGROUND Ursodeoxycholic acid(UDCA)is the first-line therapeutic agent for primary biliary cholangitis(PBC).However,a subset of patients exhibit a suboptimal response to UDCA,and reliable predictive biomarkers remain elusive.Studies have implicated plasma microRNAs(miRNAs)in the pathophysiological pro-gression of PBC,with certain miRNAs demonstrating potential as diagnostic and disease progression biomarkers.However,biomarkers capable of predicting the therapeutic efficacy of UDCA have not yet been identified.AIM To investigate differentially expressed miRNAs in PBC patients with divergent UDCA treatment responses and to explore potential biomarkers that predict treatment response in PBC.METHODS Plasma samples from treatment-naive PBC patients receiving≥1 year of standard UDCA treatment were collected.Efficacy was evaluated using the Paris I criteria.Patient samples were divided into discovery group(n=10)and validation group(n=30),with further stratification of patients into drug-resistant and drug-sensitive(DS)cohorts.Next-generation sequencing and quantitative real-time polymerase chain reaction were used to screen,functionally analyze,and validate the pre-treatment miRNA profiles of the treatment groups.RESULTS Forty-nine miRNAs were differentially expressed between the two groups before UDCA treatment(N=40).MiR-22-5p and miR-126-3p were highly expressed in the DS group before treatment(P<0.001),whereas miR-7706 exhibited a low expression(P=0.017).Post-treatment,miR-126-3p maintained low expression in the drug-resistant group(P=0.003),but showed elevated levels in the DS group(P<0.001).Logistic regression analysis identified miR-126-3p expression(odds ratio=34.32,95%confidence interval:1.95-605.40,P=0.016)as a significant factor influencing UDCA treatment response,while miR-22-5p(P=0.990)and miR-7706(P=0.157)showed no significant association.MiR-126-3p levels were negatively correlated with total bilirubin(r=-0.356,P=0.005)and immuno-globulin G levels(r=-0.311,P=0.015).The area under the receiver operating characteristic curve was 0.891(P=0.0003,95%confidence interval:0.772-1.000)with a sensitivity of 82.4%and a specificity of 84.6%.CONCLUSION Plasma miRNA expression profiles are heterogenous in patients with PBC with differential responses to UDCA therapy.MiR-126-3p demonstrates predictive potential for a suboptimal response to UDCA in patients with PBC. 展开更多
关键词 Primary biliary cholangitis MicroRNA Ursodeoxycholic acid Efficacy Early prediction
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Real-world performance of transient elastography in assessing advanced fibrosis in Chinese patients with primary biliary cholangitis 认领 引用 被引量：1
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作者 Jia-Liang Chen Yi-Xin Hou +2 位作者 Yao Liu Yu-Yong Jiang Xian-Bo Wang 《World Journal of Gastroenterology》 SCIE CAS 2025年第41期73-85,共13页
BACKGROUND There is insufficient evidence on the evaluation of liver fibrosis in Asian indivi-duals with primary biliary cholangitis(PBC)using vibration-controlled transient elastography(VCTE).AIM To assess advanced f... BACKGROUND There is insufficient evidence on the evaluation of liver fibrosis in Asian indivi-duals with primary biliary cholangitis(PBC)using vibration-controlled transient elastography(VCTE).AIM To assess advanced fibrosis(AF)using liver stiffness measurement(LSM)in Chinese patients with PBC.METHODS In total,277 Chinese patients diagnosed with PBC who underwent liver biopsy and VCTE were retrospectively included and categorized into the derivation and validation cohorts.The areas under the receiver operating characteristic curves(AUROCs)with 95%confidence intervals(CIs)were used to estimate the dia-gnostic accuracy of LSM for AF(Ludwig stage≥III).Multivariable analysis was performed using logistic regression.RESULTS In the derivation cohort,VCTE accurately detected patients with AF,achieving an AUROC of 0.93(95%CI:0.88-0.96).AF was independently predicted by LSM according to multivariable analysis.AF can be excluded and confirmed using LSM cutoffs of≤10.0 and>14.5 kPa,respectively,with a sensitivity of 0.91,negative predictive value of 0.93,specificity of 0.96,positive predictive value of 0.92,and an error rate of 7.5%.The accuracy of these values was validated in an independent cohort,achieving an AUROC of 0.97(95%CI:0.90-0.99)for AF with a sensitivity of 0.89,negative predictive value of 0.88,specificity of 0.95,positive predictive value of 0.94,and error rate of 9.0%.Compared with serum fibrosis markers,the AUROC of LSM was significantly higher in both the derivation and validation cohorts.CONCLUSION VCTE has a high accuracy for assessing AF in Chinese patients with PBC in a real-world setting. 展开更多
关键词 Primary biliary cholangitis Vibration-controlled transient elastography Fibroscan Liver stiffness measurement Liver fibrosis Chinese patients
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