2022
Disparities in Lung Transplant among Patients with Idiopathic Pulmonary Fibrosis: An Analysis of the IPF-PRO Registry
Swaminathan A, Hellkamp A, Neely M, Bender S, Paoletti L, White E, Palmer S, Whelan T, Dilling D, Baker A, Beegle S, Belperio J, Condos R, Cordova F, Culver D, Fitzgerald J, Flaherty K, Gibson K, Gulati M, Guntupalli K, Gupta N, Case A, Hotchkin D, Huie T, Kaner R, Kim H, Lancaster L, Lasky J, Lee D, Liesching T, Lipchik R, Lobo J, Luckhardt T, Mageto Y, Menon P, Morrison L, Namen A, Oldham J, Paul T, Zhang D, Porteous M, Raj R, Ramaswamy M, Russell T, Sachs P, Safdar Z, Shafazand S, Siddiqi A, Sigal B, Strek M, Suliman S, Tabak J, Walia R. Disparities in Lung Transplant among Patients with Idiopathic Pulmonary Fibrosis: An Analysis of the IPF-PRO Registry. Annals Of The American Thoracic Society 2022, 19: 981-990. PMID: 35073248, PMCID: PMC9169123, DOI: 10.1513/annalsats.202105-589oc.Peer-Reviewed Original ResearchMeSH KeywordsHumansIdiopathic Pulmonary FibrosisLung TransplantationProportional Hazards ModelsProspective StudiesRegistriesConceptsIdiopathic pulmonary fibrosisMedian ZIP code incomeZip code incomeLung transplant programsLung transplantProspective outcomes registryClinical characteristicsOutcomes RegistryPulmonary fibrosisTransplant programsSocial determinantsLung transplant centersDisease severity measuresIPF-PRO RegistryLikelihood of deathYears of ageLung transplantationPrespecified covariatesU.S. registriesDifferential associationsTransplant centersTransplant eligibilityClinical trialsTherapeutic modalitiesPatientsCough-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 ResearchMeSH KeywordsCoughDisease ProgressionHumansIdiopathic Pulmonary FibrosisLung Diseases, InterstitialQuality of LifeRegistriesConceptsInterstitial 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 modelAssociation of Circulating Proteins with Death or Lung Transplant in Patients with Idiopathic Pulmonary Fibrosis in the IPF-PRO Registry Cohort
Todd JL, Neely ML, Overton R, Mulder H, Roman J, Lasky JA, de Andrade JA, Gulati M, Huang H, Leonard TB, Hesslinger C, Noth I, Belperio JA, Flaherty KR, Palmer SM. Association of Circulating Proteins with Death or Lung Transplant in Patients with Idiopathic Pulmonary Fibrosis in the IPF-PRO Registry Cohort. Lung 2022, 200: 11-18. PMID: 35066606, PMCID: PMC8881240, DOI: 10.1007/s00408-021-00505-y.Peer-Reviewed Original ResearchMeSH KeywordsCohort StudiesHumansIdiopathic Pulmonary FibrosisLung TransplantationProteomicsRegistriesConceptsIdiopathic pulmonary fibrosisLung transplantRespiratory deathsPulmonary fibrosisClinical measuresOptimism-corrected C-indexRisk of mortalityVariable clinical courseIPF-PRO RegistryRegistry cohortComposite outcomeClinical courseMultivariable analysisUnivariable analysisPatient outcomesC-indexCirculating proteinFatal diseaseTransplantPatientsDeathFibrosisOutcomesAssociationRegistry
2020
The Pulmonary Fibrosis Foundation Patient Registry. Rationale, Design, and Methods.
Wang BR, Edwards R, Freiheit EA, Ma Y, Burg C, de Andrade J, Lancaster L, Lindell K, Nathan SD, Raghu G, Gibson K, Gulati M, Mason W, Noth I, Schmidt B, Spino C, Staszak S, Stauffer J, Wolters PJ, Cosgrove GP, Flaherty KR. The Pulmonary Fibrosis Foundation Patient Registry. Rationale, Design, and Methods. Annals Of The American Thoracic Society 2020, 17: 1620-1628. PMID: 32776789, DOI: 10.1513/annalsats.202001-035sd.Peer-Reviewed Original ResearchMeSH KeywordsAgedHumansIdiopathic Pulmonary FibrosisLung Diseases, InterstitialMaleProspective StudiesRegistriesRetrospective StudiesConceptsInterstitial lung diseasePatient RegistryClinician accessLarge multicenter registryMean diffusing capacityPositive smoking historyPercent of patientsIdiopathic pulmonary fibrosisTime of enrollmentIndividuals 18 yearsMulticenter registrySmoking historyPulmonary fibrosisSupplemental oxygenVital capacityLung diseaseMean agePatient populationAntifibrotic therapyApplicable biomarkersClinical informationDiffusing capacityPatientsClinical sitesRegistryAntifibrotic Drug Use in Patients with Idiopathic Pulmonary Fibrosis. Data from the IPF-PRO Registry
Salisbury M, Conoscenti C, Culver D, Yow E, Neely M, Bender S, Hartmann N, Palmer S, Leonard T, Baker A, Beegle S, Belperio J, Condos R, Cordova F, Culver D, Dilling D, Fitzgerald J, Flaherty K, Gibson K, Gulati M, Guntupalli K, Gupta N, Case A, Hotchkin D, Huie T, Kaner R, Kim H, Lancaster L, Lasky J, Lee D, Liesching T, Lipchik R, Lobo J, Luckhardt (formerly Joao de Andrade) T, Mageto Y, Malik N, Menon P, Morrison L, Namen A, Oldham J, Paul T, Podolanczuk A, Porteous M, Raj R, Ramaswamy M, Russell T, Sachs P, Safdar Z, Shafazand S, Siddiqi A, Sigal B, Strek M, Suliman S, Tabak J, Walia R, Whelan T. Antifibrotic Drug Use in Patients with Idiopathic Pulmonary Fibrosis. Data from the IPF-PRO Registry. Annals Of The American Thoracic Society 2020, 17: 1413-1423. PMID: 32574517, PMCID: PMC7640723, DOI: 10.1513/annalsats.201912-880oc.Peer-Reviewed Original ResearchMeSH KeywordsHumansIdiopathic Pulmonary FibrosisPharmaceutical PreparationsProspective StudiesQuality of LifeRegistriesConceptsIdiopathic pulmonary fibrosisIPF-PRO RegistrySelf-rated healthAntifibrotic medicationsEnrollment windowPulmonary fibrosisDiagnosis of IPFVital capacity percentageMajority of patientsWorse self-rated healthInterstitial lung diseaseProspective outcomes registryGreater disease severityEligible patientsU.S. registriesCarbon monoxide percentageLung biopsyPatient characteristicsMedication useOutcomes RegistrySleep apneaLung diseaseDefinite diagnosisClinical trialsFamily historyAssociations between Patient-reported Outcomes and Death or Lung transplant in IPF: Data from the IPF-PRO Registry
Case AH, Hellkamp AS, Neely ML, Bender S, Dilling DF, Gulati M, Hotchkin DL, Huie TJ, Lancaster L, Snyder LD, Conoscenti CS, Palmer S. Associations between Patient-reported Outcomes and Death or Lung transplant in IPF: Data from the IPF-PRO Registry. Annals Of The American Thoracic Society 2020, 0: 699-705. PMID: 32040340, PMCID: PMC7258421, DOI: 10.1513/annalsats.201906-437oc.Peer-Reviewed Original ResearchConceptsIdiopathic pulmonary fibrosisPatient-reported outcomesLung transplantClinical variablesSt. George's Respiratory Questionnaire total scoreSGRQ activity scoreUnderwent lung transplantClinical risk factorsPredictors of mortalityHealth-related qualityQuestionnaire total scoreIPF-PRO RegistryProspective outcomes registrySGRQ symptomsComposite outcomeExercise capacityActivity scoreOutcomes RegistryPulmonary fibrosisRisk factorsPhysical activityPatientsUnivariable modelsTransplantWorse scoresDisease Severity and Quality of Life in Patients With Idiopathic Pulmonary Fibrosis A Cross-Sectional Analysis of the IPF-PRO Registry
O’Brien E, Hellkamp A, Neely M, Swaminathan A, Bender S, Snyder L, Culver D, Conoscenti C, Todd J, Palmer S, Leonard T, investigators I, Asi W, Baker A, Beegle S, Belperio J, Condos R, Cordova F, Culver D, de Andrade J, Dilling D, Flaherty K, Glassberg M, Gulati M, Guntupalli K, Gupta N, Case A, Hotchkin D, Huie T, Kaner R, Kim H, Kreider M, Lancaster L, Lasky J, Lederer D, Lee D, Liesching T, Lipchik R, Lobo J, Mageto Y, Menon P, Morrison L, Namen A, Oldham J, Raj R, Ramaswamy M, Russell T, Sachs P, Safdar Z, Sigal B, Silhan L, Strek M, Suliman S, Tabak J, Walia R, Whelan T. Disease Severity and Quality of Life in Patients With Idiopathic Pulmonary Fibrosis A Cross-Sectional Analysis of the IPF-PRO Registry. CHEST Journal 2020, 157: 1188-1198. PMID: 31954102, DOI: 10.1016/j.chest.2019.11.042.Peer-Reviewed Original Research
2019
Peripheral blood proteomic profiling of idiopathic pulmonary fibrosis biomarkers in the multicentre IPF-PRO Registry
Todd JL, Neely ML, Overton R, Durham K, Gulati M, Huang H, Roman J, Newby LK, Flaherty KR, Vinisko R, Liu Y, Roy J, Schmid R, Strobel B, Hesslinger C, Leonard TB, Noth I, Belperio JA, Palmer SM. Peripheral blood proteomic profiling of idiopathic pulmonary fibrosis biomarkers in the multicentre IPF-PRO Registry. Respiratory Research 2019, 20: 227. PMID: 31640794, PMCID: PMC6805665, DOI: 10.1186/s12931-019-1190-z.Peer-Reviewed Original ResearchMeSH KeywordsAgedBiomarkersCohort StudiesFemaleHumansIdiopathic Pulmonary FibrosisMaleMiddle AgedProteogenomicsProteomicsRegistriesConceptsDisease severity measuresIPF-PRO RegistryProtein expressionLung diseaseC motif chemokine ligand 17Disease severitySeverity measuresBackgroundIdiopathic pulmonary fibrosisProgressive lung diseaseChemokine ligand 17Differential protein expressionVon Willebrand factorPulmonary fibrosisImmune activationPermeability-increasing proteinFibrosis biomarkersMultivariable modelHaemostatic responseLigand 17Control statusGlycoprotein thrombospondin-1IPFBiomarker candidatesThrombospondin-1Protein CPatient 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 ResearchMeSH KeywordsBiological Specimen BanksComorbidityDisease ProgressionHumansIdiopathic Pulmonary FibrosisObservational Studies as TopicRegistriesSeverity of Illness IndexConceptsIdiopathic pulmonary fibrosisPulmonary fibrosisPatient RegistryPrecision medicine approachQuality of lifeThousands of patientsClinical characteristicsRegistry databasePatient populationLarge registriesDisease progressionClinical trialsPatient profilesRare diseasePatientsRegistryDisease severityMedicine approachFibrosisDiseaseAnalysis of biospecimensBroad spectrumCurrent practiceComorbiditiesLongitudinal dataPredictors of death or lung transplant after a diagnosis of idiopathic pulmonary fibrosis: insights from the IPF-PRO Registry
Snyder L, Neely ML, Hellkamp AS, O’Brien E, de Andrade J, Conoscenti CS, Leonard T, Bender S, Gulati M, Culver DA, Kaner RJ, Palmer S, Kim HJ. Predictors of death or lung transplant after a diagnosis of idiopathic pulmonary fibrosis: insights from the IPF-PRO Registry. Respiratory Research 2019, 20: 105. PMID: 31142314, PMCID: PMC6542049, DOI: 10.1186/s12931-019-1043-9.Peer-Reviewed Original ResearchMeSH KeywordsAgedAged, 80 and overCohort StudiesFemaleFollow-Up StudiesHumansIdiopathic Pulmonary FibrosisLung TransplantationMaleMiddle AgedMortalityPredictive Value of TestsRegistriesConceptsRisk of deathLung transplantIPF-PRO RegistryPatient characteristicsDisease severityPulmonary fibrosisUS registryOxygen useBackgroundIdiopathic pulmonary fibrosisPredictors of deathIdiopathic pulmonary fibrosisVariable clinical courseAnalysis cohortLower FVCProgressive diseaseClinical courseLow DLCOMultivariable analysisMortality outcomesVital capacityMonth 30Diffusion capacityPatientsTransplantHigh mortality
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 ResearchMeSH KeywordsAgedDisease-Free SurvivalDNA, MitochondrialFemaleFibroblastsHumansIdiopathic Pulmonary FibrosisMaleConceptsIdiopathic 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
2016
Validation of the prognostic value of MMP‐7 in idiopathic pulmonary fibrosis
Tzouvelekis A, Herazo‐Maya J, Slade M, Chu J, Deiuliis G, Ryu C, Li Q, Sakamoto K, Ibarra G, Pan H, Gulati M, Antin‐Ozerkis D, Herzog EL, Kaminski N. Validation of the prognostic value of MMP‐7 in idiopathic pulmonary fibrosis. Respirology 2016, 22: 486-493. PMID: 27761978, PMCID: PMC5352520, DOI: 10.1111/resp.12920.Peer-Reviewed Original ResearchConceptsTransplant-free survivalIdiopathic pulmonary fibrosisMMP-7 concentrationsMatrix metalloproteinase-7IPF patientsCause mortalityPulmonary fibrosisHealthy controlsMultivariate Cox proportional hazards modelCox proportional hazards modelPulmonary function parametersVariable clinical courseBaseline pulmonary function parametersProportional hazards modelIPF biomarkersProgressive diseaseClinical coursePoor prognosisPrognostic valueVital capacityIndependent biomarkerLung capacityPrognostic thresholdPlasma concentrationsMortality risk
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