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 ResearchConceptsInterstitial 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 sitesRegistryAssociations 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 ResearchConceptsDisease 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 CPlasma mitochondrial DNA is associated with extrapulmonary sarcoidosis
Ryu C, Brandsdorfer C, Adams T, Hu B, Kelleher DW, Yaggi M, Manning EP, Walia A, Reeves B, Pan H, Winkler J, Minasyan M, Dela Cruz CS, Kaminski N, Gulati M, Herzog EL. Plasma mitochondrial DNA is associated with extrapulmonary sarcoidosis. European Respiratory Journal 2019, 54: 1801762. PMID: 31273041, PMCID: PMC8088542, DOI: 10.1183/13993003.01762-2018.Peer-Reviewed Original ResearchConceptsExtrapulmonary diseaseMitochondrial DNAExtracellular mtDNABAL fluidAlpha-1 antitrypsin deficiencyPlasma mitochondrial DNAPlasma of patientsAfrican AmericansExtrapulmonary sarcoidosisSarcoidosis cohortSarcoidosis subjectsScadding stageAfrican American descentClinical featuresClinical findingsGranulomatous diseaseHealthy controlsAntitrypsin deficiencyGenomic researchHigher oddsSarcoidosisAggressive phenotypeMechanistic basisDiseaseTherapeutic insightsPredictors 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 ResearchConceptsRisk 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 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
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
2010
Serum Amyloid P Therapeutically Attenuates Murine Bleomycin-Induced Pulmonary Fibrosis via Its Effects on Macrophages
Murray LA, Rosada R, Moreira AP, Joshi A, Kramer MS, Hesson DP, Argentieri RL, Mathai S, Gulati M, Herzog EL, Hogaboam CM. Serum Amyloid P Therapeutically Attenuates Murine Bleomycin-Induced Pulmonary Fibrosis via Its Effects on Macrophages. PLOS ONE 2010, 5: e9683. PMID: 20300636, PMCID: PMC2837381, DOI: 10.1371/journal.pone.0009683.Peer-Reviewed Original ResearchConceptsSerum amyloid PPulmonary fibrosisAmyloid PIPF/UIP patientsBleomycin-Induced Pulmonary FibrosisIP10/CXCL10Fibrotic lung diseaseAberrant collagen depositionRole of macrophagesM2 macrophage phenotypeUIP patientsLung diseaseM2 macrophagesCollagen depositionTissue fibrosisMacrophage activationMacrophage phenotypeFcgamma receptorsMacrophage responsePentraxin familyTherapeutic actionFibrosisMacrophagesLungTissue remodeling
2004
The World Trade Center Residents’ Respiratory Health Study: New-Onset Respiratory Symptoms and Pulmonary Function
Reibman J, Lin S, Hwang SA, Gulati M, Bowers JA, Rogers L, Berger KI, Hoerning A, Gomez M, Fitzgerald EF. The World Trade Center Residents’ Respiratory Health Study: New-Onset Respiratory Symptoms and Pulmonary Function. Environmental Health Perspectives 2004, 113: 406-411. PMID: 15811830, PMCID: PMC1278479, DOI: 10.1289/ehp.7375.Peer-Reviewed Original ResearchMeSH KeywordsAdolescentAdultAgedAir PollutantsChildChild, PreschoolCohort StudiesConstruction MaterialsCoughCross-Sectional StudiesDustDyspneaEnvironmental ExposureFemaleHumansInfantInfant, NewbornMaleMiddle AgedNew York CityResidence CharacteristicsRespiratory SoundsSeptember 11 Terrorist AttacksSpirometryConceptsNew-onset respiratory symptomsRespiratory symptomsRespiratory Health StudyNew-onset symptomsPersistent respiratory symptomsRetrospective cohort studySymptom-based questionnaireRespiratory health effectsSmall pilot studyBronchial hyperresponsivenessCohort studyPersistent symptomsPulmonary functionHealth StudyControl populationSymptomsSpirometryPilot studyHealth effectsNormal residentsMassive releaseNew York CityResidentsControl areaHyperresponsiveness