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
2020
Association of neoepitopes with disease severity and respiratory hospitalization in patients with IPF
Oldham J, Todd J, Neely M, Mulder H, Overton R, Kim H, Gulati M, Leonard T, Palmer S, Noth I, Hesslinger C. Association of neoepitopes with disease severity and respiratory hospitalization in patients with IPF. 2020, 5188. DOI: 10.1183/13993003.congress-2020.5188.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 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 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 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 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
2015
Chitinase 3–like–1 and its receptors in Hermansky-Pudlak syndrome–associated lung disease
Zhou Y, He CH, Herzog EL, Peng X, Lee CM, Nguyen TH, Gulati M, Gochuico BR, Gahl WA, Slade ML, Lee CG, Elias JA. Chitinase 3–like–1 and its receptors in Hermansky-Pudlak syndrome–associated lung disease. Journal Of Clinical Investigation 2015, 125: 3178-3192. PMID: 26121745, PMCID: PMC4563747, DOI: 10.1172/jci79792.Peer-Reviewed Original ResearchMeSH KeywordsAdipokinesAdultAnimalsApoptosisBiomarkersChitinase-3-Like Protein 1Disease Models, AnimalFemaleGlycoproteinsHermanski-Pudlak SyndromeHumansInterleukin-13 Receptor alpha2 SubunitLectinsMaleMiceMice, KnockoutPulmonary FibrosisReceptors, ImmunologicReceptors, ProstaglandinRespiratory MucosaConceptsHermansky-Pudlak syndromePulmonary fibrosisChitinase 3Pulmonary fibrosis progressionFibroproliferative repairCHI3L1 levelsFibrosis progressionLung diseaseHPS-4HPS patientsIL-13Rα2Effector functionsReceptor CRTH2Epithelial apoptosisFibrosisProtective roleDisease severityPotential biomarkersCHI3L1Major causeHPS-1Abnormal localizationPatientsCell deathSeverity
2012
Chitinase 1 Is a Biomarker for and Therapeutic Target in Scleroderma-Associated Interstitial Lung Disease That Augments TGF-β1 Signaling
Lee CG, Herzog EL, Ahangari F, Zhou Y, Gulati M, Lee CM, Peng X, Feghali-Bostwick C, Jimenez SA, Varga J, Elias JA. Chitinase 1 Is a Biomarker for and Therapeutic Target in Scleroderma-Associated Interstitial Lung Disease That Augments TGF-β1 Signaling. The Journal Of Immunology 2012, 189: 2635-2644. PMID: 22826322, PMCID: PMC4336775, DOI: 10.4049/jimmunol.1201115.Peer-Reviewed Original ResearchConceptsInterstitial lung diseaseTGF-β1 signalingPulmonary fibrosisLung diseaseTherapeutic targetScleroderma-Associated Interstitial Lung DiseaseDifferent patient cohortsTGF-β receptor 1Wild-type miceTGF-β1 effectsSSc-ILDLung involvementSSc patientsSystemic sclerosisPulmonary responseLung fibrosisPoor prognosisCHIT1 activityPatient cohortPathogenetic mechanismsReceptor expressionMurine modelingTGF-β1Disease severityPotential biomarkers
2010
TGF-beta driven lung fibrosis is macrophage dependent and blocked by Serum amyloid P
Murray LA, Chen Q, Kramer MS, Hesson DP, Argentieri RL, Peng X, Gulati M, Homer RJ, Russell T, van Rooijen N, Elias JA, Hogaboam CM, Herzog EL. TGF-beta driven lung fibrosis is macrophage dependent and blocked by Serum amyloid P. The International Journal Of Biochemistry & Cell Biology 2010, 43: 154-162. PMID: 21044893, DOI: 10.1016/j.biocel.2010.10.013.Peer-Reviewed Original ResearchConceptsSerum amyloid PAnti-fibrotic effectsLung fibrosisFibrocyte accumulationAmyloid PAberrant extracellular matrix (ECM) depositionTransgenic mouse modelM2 macrophage differentiationPleiotropic growth factorExtracellular matrix depositionAirway inflammationIPF patientsAirway remodelingPulmonary fibrosisMacrophage accumulationLung diseaseLiposomal clodronateCXCL10 expressionM2 macrophagesMonocyte responsePulmonary macrophagesMouse modelCollagen depositionPathogenic mechanismsDisease severity