2022
Cough-Specific Quality of Life Predicts Disease Progression Among Patients With Interstitial Lung Disease Data From the Pulmonary Fibrosis Foundation Patient Registry
Lee J, White E, Freiheit E, Scholand M, Strek M, Podolanczuk A, Patel N, Foundation P, Bascom R, Belloli E, Bhatt N, Bhorade S, Case A, Castriotta R, Criner G, Danoff S, De Andrade J, Desai A, Glassberg M, Glazer C, Gulati M, Gupta N, Hamblin M, Huie T, Kaner R, Kass D, Kim H, Kreider M, Lancaster L, Lasky J, Limper A, Montesi S, Mooney J, Morrison L, Nambiar A, Nathan S, Natt B, Paul T, Perez R, Podolanczuk A, Raghu G, Scholand M, Shifren A, Strek M, Todd N, Walia R, Weight S, Whelan T, Wolters P. Cough-Specific Quality of Life Predicts Disease Progression Among Patients With Interstitial Lung Disease Data From the Pulmonary Fibrosis Foundation Patient Registry. CHEST Journal 2022, 162: 603-613. PMID: 35337809, PMCID: PMC9808640, DOI: 10.1016/j.chest.2022.03.025.Peer-Reviewed Original ResearchConceptsInterstitial lung diseaseCough-specific QoLLeicester Cough QuestionnaireLung transplantationRespiratory hospitalizationsHigh riskPatient factorsLung diseaseDisease progressionLCQ scoreDisease severityMultivariable Cox regression modelsMultivariable proportional hazards modelsPatient-centered clinical outcomesBaseline disease severityGastroesophageal reflux diseaseHealth-related qualityRespiratory-related hospitalizationsCough-specific qualityIdiopathic pulmonary fibrosisPulmonary function parametersCox regression modelProportional hazards modelMultivariable proportional odds modelProportional odds model
2021
Delphi Consensus Recommendations on Management of Dosing, Adverse Events, and Comorbidities in the Treatment of Idiopathic Pulmonary Fibrosis with Nintedanib
Rahaghi F, Belperio JA, Fitzgerald J, Gulati M, Hallowell R, Highland KB, Huie TJ, Kim HJ, Kolb M, Lasky JA, Southern BD, Swigris JJ, de Andrade JA. Delphi Consensus Recommendations on Management of Dosing, Adverse Events, and Comorbidities in the Treatment of Idiopathic Pulmonary Fibrosis with Nintedanib. Therapeutic Advances In Pulmonary And Critical Care Medicine 2021, 15: 11795484211006050. PMID: 33854398, PMCID: PMC8013629, DOI: 10.1177/11795484211006050.Peer-Reviewed Original ResearchIdiopathic pulmonary fibrosisConsensus recommendationsAdverse eventsPulmonary fibrosisPrescribing informationManagement of comorbiditiesManagement of patientsAdverse event managementClinical trial dataEvidence-based literatureReal-world evidenceCytochrome P450 3A4Delphi consensus recommendationsConcomitant medicationsLiver dysfunctionTolerability issuesConcomitant administrationGastrointestinal effectsDisease progressionNintedanibComorbiditiesAppropriate managementTrial dataPatientsP450 3A4
2019
Patient Registries in Idiopathic Pulmonary Fibrosis
Culver DA, Behr J, Belperio JA, Corte TJ, de Andrade JA, Flaherty KR, Gulati M, Huie TJ, Lancaster LH, Roman J, Ryerson CJ, Kim HJ. Patient Registries in Idiopathic Pulmonary Fibrosis. American Journal Of Respiratory And Critical Care Medicine 2019, 200: 160-167. PMID: 31034241, PMCID: PMC6635784, DOI: 10.1164/rccm.201902-0431ci.Peer-Reviewed Original ResearchConceptsIdiopathic pulmonary fibrosisPulmonary fibrosisPatient RegistryPrecision medicine approachQuality of lifeThousands of patientsClinical characteristicsRegistry databasePatient populationLarge registriesDisease progressionClinical trialsPatient profilesRare diseasePatientsRegistryDisease severityMedicine approachFibrosisDiseaseAnalysis of biospecimensBroad spectrumCurrent practiceComorbiditiesLongitudinal dataCirculating Mitochondrial DNA Is Associated with Fibroblast Activation and Disease Progression in Scleroderma Associated Interstitial Lung Disease
Ryu C, Sun H, Winkler J, Meena S, Walia A, Minasyan M, Brandsdorfer C, Gulati M, Peng X, Herzog E. Circulating Mitochondrial DNA Is Associated with Fibroblast Activation and Disease Progression in Scleroderma Associated Interstitial Lung Disease. 2019, a7219-a7219. DOI: 10.1164/ajrccm-conference.2019.199.1_meetingabstracts.a7219.Peer-Reviewed Original Research
2017
Extracellular Mitochondrial DNA Is Generated by Fibroblasts and Predicts Death in Idiopathic Pulmonary Fibrosis
Ryu C, Sun H, Gulati M, Herazo-Maya J, Chen Y, Osafo-Addo A, Brandsdorfer C, Winkler J, Blaul C, Faunce J, Pan H, Woolard T, Tzouvelekis A, Antin-Ozerkis DE, Puchalski JT, Slade M, Gonzalez AL, Bogenhagen DF, Kirillov V, Feghali-Bostwick C, Gibson K, Lindell K, Herzog RI, Dela Cruz CS, Mehal W, Kaminski N, Herzog EL, Trujillo G. Extracellular Mitochondrial DNA Is Generated by Fibroblasts and Predicts Death in Idiopathic Pulmonary Fibrosis. American Journal Of Respiratory And Critical Care Medicine 2017, 196: 1571-1581. PMID: 28783377, PMCID: PMC5754440, DOI: 10.1164/rccm.201612-2480oc.Peer-Reviewed Original ResearchConceptsIdiopathic pulmonary fibrosisNormal human lung fibroblastsExtracellular mitochondrial DNABronchoalveolar lavageIPF fibroblastsPulmonary fibrosisInnate immune ligandsEvent-free survivalSmooth muscle actin expressionMtDNA concentrationsSmooth muscle actin-expressing myofibroblastsGrowth factor-β1Muscle actin expressionHuman lung fibroblastsTGF-β1 stimulationExtracellular mtDNAIPF cohortClinical outcomesControl subjectsDisease progressionGlycolytic reprogrammingSoluble mediatorsTGF-β1Factor-β1Immune ligands
2014
Chitinase 3–Like 1 Suppresses Injury and Promotes Fibroproliferative Responses in Mammalian Lung Fibrosis
Zhou Y, Peng H, Sun H, Peng X, Tang C, Gan Y, Chen X, Mathur A, Hu B, Slade MD, Montgomery RR, Shaw AC, Homer RJ, White ES, Lee CM, Moore MW, Gulati M, Lee CG, Elias JA, Herzog EL. Chitinase 3–Like 1 Suppresses Injury and Promotes Fibroproliferative Responses in Mammalian Lung Fibrosis. Science Translational Medicine 2014, 6: 240ra76. PMID: 24920662, PMCID: PMC4340473, DOI: 10.1126/scitranslmed.3007096.Peer-Reviewed Original ResearchConceptsIdiopathic pulmonary fibrosisCHI3L1 levelsChitinase 3Lungs of patientsAlternative macrophage activationLevel of apoptosisAcute exacerbationFibroproliferative repairLung transplantationDisease exacerbationInjury phaseAmbulatory patientsEpithelial injuryPulmonary fibrosisIPF populationLung fibrosisMacrophage accumulationCHI3L1 expressionFibrotic phaseDisease progressionProfibrotic roleFibroproliferative responseMacrophage activationMyofibroblast transformationProtective role
2012
Semaphorin 7a+ Regulatory T Cells Are Associated with Progressive Idiopathic Pulmonary Fibrosis and Are Implicated in Transforming Growth Factor-β1–induced Pulmonary Fibrosis
Reilkoff RA, Peng H, Murray LA, Peng X, Russell T, Montgomery R, Feghali-Bostwick C, Shaw A, Homer RJ, Gulati M, Mathur A, Elias JA, Herzog EL. Semaphorin 7a+ Regulatory T Cells Are Associated with Progressive Idiopathic Pulmonary Fibrosis and Are Implicated in Transforming Growth Factor-β1–induced Pulmonary Fibrosis. American Journal Of Respiratory And Critical Care Medicine 2012, 187: 180-188. PMID: 23220917, PMCID: PMC3570653, DOI: 10.1164/rccm.201206-1109oc.Peer-Reviewed Original ResearchConceptsIdiopathic pulmonary fibrosisRegulatory T cellsProgressive idiopathic pulmonary fibrosisSEMA 7ATGF-β1Pulmonary fibrosisLung fibrosisT cellsMurine lungIL-10Bone marrow-derived cellsAdoptive transfer approachT-cell mediatorsMarrow-derived cellsTransforming Growth Factor-β1Murine lung fibrosisGrowth factor-β1Lung CD4Adoptive transferIL-17AIL-4Disease progressionSemaphorin 7ACD4Mouse model