2024
Long-term Air Pollution Exposure Is Associated With Increased Severity of Idiopathic Pulmonary Fibrosis in the IPF-PRO Registry
Sack C, Wojdyla D, Macmurdo M, Gassett A, Kaufman J, Raghu G, Redlich C, Li P, Olson A, Leonard T, Todd J, Neely M, Snyder L, Gulati M, on behalf of the IPF-PRO Registry investigators. Long-term Air Pollution Exposure Is Associated With Increased Severity of Idiopathic Pulmonary Fibrosis in the IPF-PRO Registry. 2024, a1798-a1798. DOI: 10.1164/ajrccm-conference.2024.209.1_meetingabstracts.a1798.Peer-Reviewed Original Research
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 ResearchConceptsIdiopathic 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 modalitiesPatientsAssociation 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 ResearchConceptsIdiopathic 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
Antifibrotic 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 ResearchConceptsIdiopathic 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 scores
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 CPredictors 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
2018
Predictors of death or transplant in patients with idiopathic pulmonary fibrosis in the IPF-PRO Registry
Snyder L, Neely M, Hellkamp A, O'Brien E, Flaherty K, De Andrade J, Conoscenti C, Leonard T, Bender S, Gulati M, Culver D, Kaner R, Palmer S, Kim H. Predictors of death or transplant in patients with idiopathic pulmonary fibrosis in the IPF-PRO Registry. 2018, pa2196. DOI: 10.1183/13993003.congress-2018.pa2196.Peer-Reviewed Original Research