2025
Changes in Lung Function and Mortality Risk in Patients With Idiopathic Pulmonary Fibrosis
Oldham J, Neely M, Wojdyla D, Gulati M, Li P, Patel D, Palmer S, Todd J, Investigators I. Changes in Lung Function and Mortality Risk in Patients With Idiopathic Pulmonary Fibrosis. CHEST Journal 2025 PMID: 40020995, DOI: 10.1016/j.chest.2025.02.018.Peer-Reviewed Original ResearchIdiopathic pulmonary fibrosisLung transplantationLung function declinePulmonary fibrosisAssociated with 1.8-foldProgressive fibrosing interstitial lung diseaseMedian follow-up timeFibrosing interstitial lung diseaseFollow-up timeInterstitial lung diseaseCox proportional hazards modelsRisk of deathFunctional declineRisk of mortalityProportional hazards modelAntifibrotic treatmentThreshold of declineAssociated with lung function declineOutcomes RegistryLung diseaseEnrolling centersDLCOSmoking statusDiffusing capacityLung function
2024
Pamrevlumab for Idiopathic Pulmonary Fibrosis
Raghu G, Richeldi L, Fernández Pérez E, De Salvo M, Silva R, Song J, Ogura T, Xu Z, Belloli E, Zhang X, Seid L, Poole L, Bowler S, Corte T, Holmes M, Thien F, Wheatley J, Choi S, Chung M, Jeong S, Kim Y, Lee E, Lee H, Park C, Park J, Park J, Chi-Leung Lam D, Chan M, Lee K, Cao J, Chen J, Chen R, Dai H, Fu X, Liang Z, Luo Q, Shi G, Tong Z, Wang L, Yang S, Yu H, Zhang H, Zhang J, Zhao H, Wang W, Meng Y, Peng H, Ramaswamy M, Hamblin M, Fitzgerald J, Gupta N, Dematte J, Veeraraghavan S, O’Brien T, Luckhardt T, Lancaster L, Kokoszynska M, Ettinger N, Kaelin T, Siddiqi A, Collins B, Scholand M, Antin-Ozerkis D, Hyun K, Harden C, Averill F, Mallea J, Bascom R, Seeram V, Hajari Case A, Britt E, Shea B, Criner G, Gotfried M, Mageto Y, El Bayadi S, Reichner C, Mooney J, Hotchkin D, Abrencillo R, Boente R, Lee J, Betensley A, Jeganathan N, Walia R, Albertson T, Rosas I, Puppala D, Abraham L, Enelow R, Bhatt N, Bandyopadhyay D, Elias P, Bergna M, Garcia G, De Stefano G, Wehbe L, Chirino A, Rojas R, Otaola M, Miranda G, Florenzano M, Silva Orellana R, Glasinovich V, Shangina O, Nikishenkov A, Kuzubova N. Pamrevlumab for Idiopathic Pulmonary Fibrosis. JAMA 2024, 332: 380-389. PMID: 38762797, PMCID: PMC11304118, DOI: 10.1001/jama.2024.8693.Peer-Reviewed Original ResearchIdiopathic pulmonary fibrosisBaseline to weekPulmonary fibrosisAdverse eventsBetween-group differencesPrimary outcomePhase 3 randomized clinical trialSecondary outcomesTreatment-related adverse eventsProgression of idiopathic pulmonary fibrosisPhase 2 trialAssociated with adverse eventsRate of lung function declineSubstantial adverse eventsAbsolute changeStatistically significant between-group differencesPatient-reported symptomsLung function declineSignificant between-group differencesFollow-up encountersPatient-reported outcomesPlacebo groupAntifibrotic treatmentPamrevlumabClinical trialsPulmonary Fibrosis Stakeholder Summit: A Joint NHLBI, Three Lakes Foundation, and Pulmonary Fibrosis Foundation Workshop Report
Montesi S, Gomez C, Beers M, Brown R, Chattopadhyay I, Flaherty K, Garcia C, Gomperts B, Hariri L, Hogaboam C, Jenkins R, Kaminski N, Kim G, Königshoff M, Kolb M, Kotton D, Kropski J, Lasky J, Magin C, Maher T, McCormick M, Moore B, Nickerson-Nutter C, Oldham J, Podolanczuk A, Raghu G, Rosas I, Rowe S, Schmidt W, Schwartz D, Shore J, Spino C, Craig J, Martinez F. Pulmonary Fibrosis Stakeholder Summit: A Joint NHLBI, Three Lakes Foundation, and Pulmonary Fibrosis Foundation Workshop Report. American Journal Of Respiratory And Critical Care Medicine 2024, 209: 362-373. PMID: 38113442, PMCID: PMC10878386, DOI: 10.1164/rccm.202307-1154ws.Peer-Reviewed Original ResearchConceptsSlow lung function declinePulmonary fibrosisLung function declineField of pulmonary fibrosisRisk factorsStudy pulmonary fibrosisProgressive pulmonary fibrosisIdiopathic pulmonary fibrosisPulmonary Fibrosis FoundationIdentification of risk factorsFunctional declineElucidation of disease mechanismsClinical trial designDisease risk factorsFibrosisImprove diagnosisTrial designBiomarker discoveryDisease mechanismsPulmonaryMedicationMorbidityTherapy
2023
Bacteriophage Therapy for Pan-Drug-Resistant Pseudomonas aeruginosa in Two Persons With Cystic Fibrosis
Hahn A, Sami I, Chaney H, Koumbourlis A, Del Valle Mojica C, Cochrane C, Chan B, Koff J. Bacteriophage Therapy for Pan-Drug-Resistant Pseudomonas aeruginosa in Two Persons With Cystic Fibrosis. Journal Of Investigative Medicine High Impact Case Reports 2023, 11: 23247096231188243. PMID: 37515541, PMCID: PMC10387758, DOI: 10.1177/23247096231188243.Peer-Reviewed Original ResearchConceptsPulmonary exacerbationsCF transmembrane conductance regulator geneTransmembrane conductance regulator geneChronic bacterial colonizationRecurrent lung infectionsStructural lung damagePediatric clinical trialsLung function declineLung inflammationTreatment modalitiesLung infectionCystic fibrosisLung damageClinical trialsTherapeutic strategiesAntibiotic resistanceBacterial colonizationMonogenic diseasesBacteriophage therapyRegulated genesViscous secretionsReduced survivalFunctional declineExacerbationBacteriophage
2022
Nebulization of risedronate alleviates airway obstruction and inflammation of chronic obstructive pulmonary diseases via suppressing prenylation-dependent RAS/ERK/NF-κB and RhoA/ROCK1/MLCP signaling
Liu D, Xu W, Tang Y, Cao J, Chen R, Wu D, Chen H, Su B, Xu J. Nebulization of risedronate alleviates airway obstruction and inflammation of chronic obstructive pulmonary diseases via suppressing prenylation-dependent RAS/ERK/NF-κB and RhoA/ROCK1/MLCP signaling. Respiratory Research 2022, 23: 380. PMID: 36575527, PMCID: PMC9795678, DOI: 10.1186/s12931-022-02274-5.Peer-Reviewed Original ResearchConceptsAirway smooth muscle cellsAirway obstructionB2 agonistsObstructive pulmonary diseaseLung inflammationCOPD ratsInflammation of chronic obstructive pulmonary diseaseExpression of B2 receptorsLung depositionPulmonary diseaseTumor necrosis factor-alphaFirst-line treatmentNecrosis factor-alphaTreating COPD patientsChronic obstructive pulmonary diseaseSmooth muscle cellsTreatment of COPDBackgroundChronic obstructive pulmonary diseaseNitrogen-Containing BisphosphonatesProgression of COPDLung function declineLong-term useRisedronate treatmentASMCs relaxationAntimuscarinic drugs
2021
SPLUNC1: a novel marker of cystic fibrosis exacerbations
Khanal S, Webster M, Niu N, Zielonka J, Nunez M, Chupp G, Slade MD, Cohn L, Sauler M, Gomez JL, Tarran R, Sharma L, Dela Cruz CS, Egan M, Laguna T, Britto CJ. SPLUNC1: a novel marker of cystic fibrosis exacerbations. European Respiratory Journal 2021, 58: 2000507. PMID: 33958427, PMCID: PMC8571118, DOI: 10.1183/13993003.00507-2020.Peer-Reviewed Original ResearchConceptsAcute pulmonary exacerbationsSPLUNC1 levelsCystic fibrosisClinical outcomesCF participantsLong-term disease controlNasal epithelium clone 1Cystic fibrosis exacerbationsHigher AE riskLung function declineCytokines interleukin-1βTumor necrosis factorAE riskClinical worseningPulmonary exacerbationsStable patientsLung functionAirway clearanceFunction declineSputum collectionAcute inflammationInflammatory cytokinesMicrobiology findingsCF careClinical managementNO2 exposure and lung function decline in a cohort of adults in Mysore, India
Nori-Sarma A, Thimmulappa R, Venkataraman G, Warren J, Berman J, Whittaker S, Kulick E, Wellenius G, Mahesh P, Bell M. NO2 exposure and lung function decline in a cohort of adults in Mysore, India. Environmental Research Communications 2021, 3: 055001. DOI: 10.1088/2515-7620/abf2dd.Peer-Reviewed Original ResearchLung function declineAir pollution exposureLung functionFunction declineLong-term nitrogen dioxide exposurePollution exposureWorse lung functionCohort of adultsInterquartile range increaseNitrogen dioxide exposureLow-income adultsMiddle-income countriesPotential confoundersRespiratory healthHigh riskHealthy individualsLongitudinal health dataLinear mixed effects modelsStandardized interviewHealthy participantsRelevant studiesNO2 exposureMixed effects modelsPoor populationCohort
2018
Influence of viral infection on the relationships between airway cytokines and lung function in asthmatic children
Lewis T, Metitiri E, Mentz G, Ren X, Carpenter A, Goldsmith A, Wicklund K, Eder B, Comstock A, Ricci J, Brennan S, Washington G, Owens K, Mukherjee B, Robins T, Batterman S, Hershenson M. Influence of viral infection on the relationships between airway cytokines and lung function in asthmatic children. Respiratory Research 2018, 19: 228. PMID: 30463560, PMCID: PMC6249926, DOI: 10.1186/s12931-018-0922-9.Peer-Reviewed Original ResearchConceptsViral infectionLung functionAbsence of viral infectionIL-13IL-4Viral-induced asthma exacerbationsRespiratory illnessRespiratory viral infectionsAssociated with increased cytokinesPro-inflammatory cytokinesLung function declineSelf-reported respiratory illnessInfluence of viral infectionAirway cytokinesMDA5 mRNACXCL10 mRNACytokine levelsAirway responsivenessNasal lavageAirflow obstructionAssociated with FVCAsthma exacerbationsAssociated with decreased lung functionInflammatory responseNasal samplesSafety and efficacy of pirfenidone in patients carrying telomerase complex mutation
Justet A, Thabut G, Manali E, Molina M, Kannengiesser C, Cadranel J, Cottin V, Gondouin A, Nunes H, Magois E, Tromeur C, Prevot G, Papiris S, Marchand-Adam S, Gamez A, Reynaud-Gaubert M, Wemeau L, Crestani B, Borie R. Safety and efficacy of pirfenidone in patients carrying telomerase complex mutation. European Respiratory Journal 2018, 51: 1701875. PMID: 29449422, DOI: 10.1183/13993003.01875-2017.Peer-Reviewed Original ResearchConceptsFamilial pulmonary fibrosisIdiopathic pulmonary fibrosisEfficacy of pirfenidoneMutation carriersForced Vital CapacityRetrospective studyPulmonary fibrosisTreatment of idiopathic pulmonary fibrosisReduced FVC declineProgression-free survivalMulticentre retrospective studyEffect of pirfenidoneLung function declineTERC mutationsFVC declineMultidisciplinary diagnosisAssociated with bloodPatient carriersFrequent mutationsPirfenidoneSynthetic androgenDiffusing capacitySkin disordersPatientsVital capacity
2017
Consensus network analysis reveals pathways associated with lung function decline in both COPD and IPF
Mcdonough J, Vanaudenaerde B, Wuyts W, Kaminski N. Consensus network analysis reveals pathways associated with lung function decline in both COPD and IPF. 2017, pa3484. DOI: 10.1183/1393003.congress-2017.pa3484.Peer-Reviewed Original ResearchLung function declineFunction decline
2016
NK cell activating receptor ligand expression in lymphangioleiomyomatosis is associated with lung function decline
Osterburg AR, Nelson RL, Yaniv BZ, Foot R, Donica W, Nashu MA, Liu H, Wikenheiser-Brokamp KA, Moss J, Gupta N, McCormack FX, Borchers MT. NK cell activating receptor ligand expression in lymphangioleiomyomatosis is associated with lung function decline. JCI Insight 2016, 1: e87270. PMID: 27734028, PMCID: PMC5053147, DOI: 10.1172/jci.insight.87270.Peer-Reviewed Original ResearchConceptsLung function declineNK cellsLAM pathogenesisFunction declineNeoplastic smooth muscle cellsNK cell activating receptorsNKG2D surface expressionRare lung diseaseInnate immune systemSmooth muscle cellsExpiratory volumePulmonary functionLAM patientsLung diseaseActivating receptorsLAM lesionsImmune surveillanceImmune systemBinding protein 2Ligand expressionPulmonary nodulesLymphangioleiomyomatosisMuscle cellsPatientsCyst formationUsefulness of Cyclophosphamide Pulse Therapy in Interstitial Lung Diseases
Schupp J, Köhler T, Müller-Quernheim J. Usefulness of Cyclophosphamide Pulse Therapy in Interstitial Lung Diseases. Respiration 2016, 91: 296-301. PMID: 27082957, DOI: 10.1159/000445031.Peer-Reviewed Original ResearchConceptsNon-specific interstitial pneumoniaInterstitial lung diseaseLymphocytic interstitial pneumoniaCyclophosphamide pulse therapyLung functionPulse therapyInterstitial pneumoniaLung diseaseRheumatoid arthritis-associated interstitial lung diseaseIdiopathic non-specific interstitial pneumoniaFibrotic interstitial lung diseaseProgressive lung function declineBetter survival outcomesLung function declineIdiopathic pulmonary fibrosisBronchoalveolar lavage cytologyGroup of disordersILD patientsMost patientsSystemic sclerosisTotal cohortFunction declineLavage cytologyPulmonary fibrosisCyclophosphamide treatment
2015
Longitudinal assessment of lung function decline in the occupational setting
Redlich CA, Tarlo SM. Longitudinal assessment of lung function decline in the occupational setting. Current Opinion In Allergy And Clinical Immunology 2015, 15: 145-149. PMID: 25961387, DOI: 10.1097/aci.0000000000000153.Peer-Reviewed Original ResearchConceptsLongitudinal lung functionLung functionLongitudinal spirometryMedical surveillanceFlavorings-related lung diseaseHazardous work exposuresLung function declineOccupational respiratory diseasesRecent longitudinal studiesFunction declineEarly diseaseLung diseaseOccupational exposureRespiratory diseaseWork exposureSpirometryPreventive interventionsLongitudinal assessmentFurther evaluationOccupational settingsDiseaseLongitudinal studyCliniciansExposureRecent studies
2011
Hemolytic Phospholipase C Inhibition Protects Lung Function during Pseudomonas aeruginosa Infection
Wargo M, Gross M, Rajamani S, Allard J, Lundblad L, Allen G, Vasil M, Leclair L, Hogan D. Hemolytic Phospholipase C Inhibition Protects Lung Function during Pseudomonas aeruginosa Infection. American Journal Of Respiratory And Critical Care Medicine 2011, 184: 345-354. PMID: 21562128, PMCID: PMC3175536, DOI: 10.1164/rccm.201103-0374oc.Peer-Reviewed Original ResearchConceptsLung functionP. aeruginosa infectionAeruginosa infectionSignificant lung function impairmentLung function impairmentLung function declineBronchoalveolar lavage fluidPseudomonas aeruginosa infectionChronic lung infectionVirulence factorsP. aeruginosaP. aeruginosa virulence factorsPulmonary surfactant functionSingle virulence factorFunction declineMechanical ventilationFunction impairmentLavage fluidRespiratory endpointsSurfactant dysfunctionComputer-controlled ventilatorLung infectionAeruginosa virulence factorsStrain infectionLung physiology
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