2024
Fecal deoxycholic acid associates with diet, intestinal microbes, and total bilirubin in primary sclerosing cholangitis
Chan C, Lemos M, Finnegan P, Gagnon W, Dean R, Yazdanafar M, Zepeda J, Vohl M, Trauner M, Korzenik J, Barbier O, Marco M, Bowlus C. Fecal deoxycholic acid associates with diet, intestinal microbes, and total bilirubin in primary sclerosing cholangitis. JHEP Reports 2024, 6: 101188. DOI: 10.1016/j.jhepr.2024.101188.Peer-Reviewed Original ResearchPrimary sclerosing cholangitisMarkers of BA synthesisFecal deoxycholic acidInflammatory bowel diseaseCholestatic liver diseaseFarnesoid X receptorFarnesoid X receptor activationSclerosing cholangitisDisease progressionFecal bile acidsTotal bilirubinDuct primary sclerosing cholangitisBile acidsLiver diseaseMarker of clinical outcomeBA synthesisChronic cholestatic liver diseaseRRNA gene sequencesUrsodeoxycholic acid useDeoxycholic acidAbundance of BlautiaMarkers of disease progressionVariable disease progressionAssociated with total bilirubinSerum total bilirubinAnti-integrin αvβ6 autoantibodies are increased in PSC patients with concomitant IBD and correlate with liver disease severity
Bloemen H, Livanos A, Martins A, Dean R, Bravo A, Bourgonje A, Tankelevich M, Herb J, Cho J, Santos A, Rodrigues C, Petralia F, Colombel J, Bowlus C, Schiano T, Torres J, Levy C, Mehandru S. Anti-integrin αvβ6 autoantibodies are increased in PSC patients with concomitant IBD and correlate with liver disease severity. Clinical Gastroenterology And Hepatology 2024 PMID: 39490950, DOI: 10.1016/j.cgh.2024.10.005.Peer-Reviewed Original ResearchPrimary sclerosing cholangitis patientsPrimary sclerosing cholangitisLiver disease severityConcomitant inflammatory bowel diseaseInflammatory bowel diseaseHealthy controlsEnzyme-linked immunosorbent assayDisease severityAssociated with liver disease severityUlcerative colitisAssociated with markers of systemic inflammationMayo PSC risk scoreMarkers of systemic inflammationMarkers of liver disease severityLiver stiffness measurementMultivariate linear regression analysisAlkaline phosphatase levelsAssociated with markersInflammatory bowel disease subtypePSC patientsSclerosing cholangitisInflammation panelSystemic inflammationPSC-UCPSC-IBDSerologic extracellular matrix remodeling markers are related to fibrosis stage and prognosis in a phase 2b trial of simtuzumab in patients with primary sclerosing cholangitis
Thorburn D, Leeming D, Barchuk W, Wang Y, Lu X, Malkov V, Ito K, Bowlus C, Levy C, Goodman Z, Karsdal M, Muir A, Xu J. Serologic extracellular matrix remodeling markers are related to fibrosis stage and prognosis in a phase 2b trial of simtuzumab in patients with primary sclerosing cholangitis. Hepatology Communications 2024, 8: e0467. PMID: 38967589, PMCID: PMC11227354, DOI: 10.1097/hc9.0000000000000467.Peer-Reviewed Original ResearchConceptsPrimary sclerosing cholangitisELF scoreAdvanced fibrosisFibrosis stageFibrosis progressionClinical eventsSclerosing cholangitisExtracellular matrix remodeling markersRemodeling markersPredictors of disease severityPhase 2b trialBaseline to weekPRO-C3Progression to cirrhosisWilcoxon rank sum testMarker of collagen formationType III procollagenModerate diagnostic abilityDiagnosing fibrosis stagesRank sum testSerum markersNoninvasive predictorPrognostic markerModerate prognosisMedian changeProtocol for the development of a core outcome set for clinical trials in primary sclerosing cholangitis
Hussain N, Ma C, Hirschfield G, Walmsley M, Hanford P, Vesterhus M, Kowdley K, Bergquist A, Ponsioen C, Levy C, Assis D, Schramm C, Bowlus C, Trauner M, Aiyegbusi O, Jairath V, Trivedi P. Protocol for the development of a core outcome set for clinical trials in primary sclerosing cholangitis. BMJ Open 2024, 14: e080143. PMID: 38926149, PMCID: PMC11216047, DOI: 10.1136/bmjopen-2023-080143.Peer-Reviewed Original ResearchConceptsPrimary sclerosing cholangitisClinical trialsSclerosing cholangitisCore outcome setImmune-mediated liver diseasesPrimary sclerosing cholangitis treatmentOutcome measuresEvaluate novel therapiesSlow disease progressionPatient-reported outcome measuresMedical therapyNovel therapiesLiver fibrosisDisease progressionHistological assessmentLiver diseaseInternational two-round Delphi surveyImaging-based biomarkersIntervention trialsTherapyConsensus meetingHealthcare payersCholangitisSemistructured qualitative interviewsTwo-round Delphi surveyLBP-039 Oral alpha-v/beta-6 and alpha-v/beta-1 integrin inhibitor bexotegrast in primary sclerosing cholangitis: updated 12-week interim safety and efficacy analysis of the INTEGRIS-PSC phase 2a trial
Trauner M, Kowdley K, Hirschfield G, Lefebvre E, Decaris M, Schaub J, Clark A, Lin M, An M, Balasubramanyam A, Barnes C, Pencek R, Thorburn D, Montano-Loza A, Bowlus C, Schramm C, Levy C, Trivedi P. LBP-039 Oral alpha-v/beta-6 and alpha-v/beta-1 integrin inhibitor bexotegrast in primary sclerosing cholangitis: updated 12-week interim safety and efficacy analysis of the INTEGRIS-PSC phase 2a trial. Journal Of Hepatology 2024, 80: s97-s98. DOI: 10.1016/s0168-8278(24)00606-8.Peer-Reviewed Original ResearchPrimary sclerosing cholangitisPhase 2a trialEfficacy analysisInterim safetySclerosing cholangitisCholangitisPatient-focused drug development in primary sclerosing cholangitis: Insights on patient priorities and involvement in clinical trials
Li M, Pai R, Gomel R, Vyas M, Callif S, Hatchett J, Bowlus C, Lai J. Patient-focused drug development in primary sclerosing cholangitis: Insights on patient priorities and involvement in clinical trials. Hepatology Communications 2024, 8: e0433. PMID: 38727680, PMCID: PMC11093571, DOI: 10.1097/hc9.0000000000000433.Peer-Reviewed Original ResearchConceptsPrimary sclerosing cholangitisClinical trialsSclerosing cholangitisDrug trialsTrial participantsMultivariate logistic regressionAssociated with willingnessInflammatory bowel diseaseLong-term riskReferral of patientsDrug developmentTherapy trialsDisease progressionTrial interestBowel diseasePatient-focused drug developmentTreatment trialsPatientsBiopsy requirementsPruritusPatient interestPredictors of patients' willingnessLogistic regressionCholangitisPatients' willingnessPer-oral cholangioscopy in patients with primary sclerosing cholangitis: a 12-month follow-up study
Mohamed R, Tejaswi S, Aabakken L, Ponsioen C, Bowlus C, Adler D, Forbes N, Paulsen V, Voermans R, Urayama S, Peetermans J, Rousseau M, Eksteen B. Per-oral cholangioscopy in patients with primary sclerosing cholangitis: a 12-month follow-up study. Endoscopy International Open 2024, 12: e237-e244. PMID: 38362361, PMCID: PMC10869209, DOI: 10.1055/a-2236-7557.Peer-Reviewed Original ResearchEndoscopic retrograde cholangiographySerious adverse eventsPrimary sclerosing cholangitisLiver transplantationIndex procedurePSC patientsSclerosing cholangitisOral cholangioscopyRepeat endoscopic retrograde cholangiographyPrior liver transplantationCases of cholangiocarcinomaCCA incidencePrimary endpointSecondary endpointsAdverse eventsBiopsy findingsConsecutive patientsRetrograde cholangiographyAdditional patientsCytology findingsRepeat proceduresMean ageTherapeutic maneuversBenign cytologyCCA diagnosisAfrican American race does not confer an increased risk of clinical events in patients with primary sclerosing cholangitis.
Yazdanfar M, Zepeda J, Dean R, Wu J, Levy C, Goldberg D, Lammert C, Prenner S, Reddy K, Pratt D, Forman L, Assis D, Lytvyak E, Montano-Loza A, Gordon S, Carey E, Ahn J, Schlansky B, Korzenik J, Karagozian R, Hameed B, Chandna S, Yu L, Bowlus C. African American race does not confer an increased risk of clinical events in patients with primary sclerosing cholangitis. Hepatology Communications 2024, 8 PMID: 38285883, PMCID: PMC10830082, DOI: 10.1097/hc9.0000000000000366.Peer-Reviewed Original ResearchConceptsPrimary sclerosing cholangitisTransplant-free survivalInflammatory bowel diseaseHepatic decompensationNon-Hispanic whitesSclerosing cholangitisIncreased risk of clinical eventsNatural history of primary sclerosing cholangitisAssociated with transplant-free survivalHistory of primary sclerosing cholangitisAssociated with hepatic decompensationBowel diseaseProgression to hepatic decompensationRisk of clinical eventsDecompensation-free survivalMayo risk scoreAbnormal liver testsPerformance of prognostic modelsAfrican American raceRates of inflammatory bowel diseaseDeath/liver transplantationAA patientsLiver testsDiagnostic delayAA raceChapter 43 Primary sclerosing cholangitis and IgG4-associated cholangitis
Bowlus C. Chapter 43 Primary sclerosing cholangitis and IgG4-associated cholangitis. 2024, 845-868. DOI: 10.1016/b978-0-443-23947-2.00020-5.Peer-Reviewed Original ResearchIgG4-associated cholangitisPrimary sclerosing cholangitisInflammatory bowel diseaseAssociated with inflammatory bowel diseaseFeatures of primary sclerosing cholangitisResponse to corticosteroidsSclerosing cholangitisClinical featuresInflammatory disordersBiliary treeBowel diseaseNatural historyOlder menCholangitisYoung adults
2023
EPH110 Structured Literature Review of Evidence on the Clinical, Humanistic, and Economic Burden of Primary Sclerosing Cholangitis
Levy C, Bowlus C, Kachru N, Ammirabile G, Walker S, Alleman C, Kowdley K. EPH110 Structured Literature Review of Evidence on the Clinical, Humanistic, and Economic Burden of Primary Sclerosing Cholangitis. Value In Health 2023, 26: s184. DOI: 10.1016/j.jval.2023.03.992.Peer-Reviewed Original Research