2024
Development and evaluation of a questionnaire to capture environmental and occupational inhalational exposures in adults with fibrotic interstitial lung disease
Swaminathan A, McFatrich M, Mkumba L, Wright L, Redlich C, Snyder L, Reeve B, Patel D, Gulati M. Development and evaluation of a questionnaire to capture environmental and occupational inhalational exposures in adults with fibrotic interstitial lung disease. Respiratory Research 2024, 25: 372. PMID: 39407223, PMCID: PMC11481565, DOI: 10.1186/s12931-024-03000-z.Peer-Reviewed Original ResearchConceptsCognitive interviewsContent validityExposure questionnaireMultidisciplinary teamSemi-structured interview guideEvidence of content validityFibrosing ILDsInterstitial lung diseaseRelevant exposuresFibrotic interstitial lung diseaseClinically relevant exposuresTrained interviewersInterview guideAssessment QuestionnaireQuestionnaireInterviewsLung diseaseAdultsTeamClinicPatientsSource of misunderstandingOccupational inhalation exposureValidityDisease
2023
“Long Haulers”
Lutchmansingh D, Sevilla J, Possick J, Gulati M. “Long Haulers”. Seminars In Respiratory And Critical Care Medicine 2023, 44: 130-142. PMID: 36646091, DOI: 10.1055/s-0042-1759568.Peer-Reviewed Reviews, Practice Guidelines, Standards, and Consensus StatementsConceptsPost-COVID conditionsAcute severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infectionAcute respiratory syndrome coronavirus 2 infectionSevere acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infectionSyndrome coronavirus 2 infectionSARS-CoV-2 infectionChronic COVID-19Long-haul COVIDOngoing interventional trialsLong-term morbidityCoronavirus disease 2019Multiple organ systemsInterventional trialsLong COVIDTreatment algorithmNeuropsychiatric sequelaePostacute sequelaeRisk factorsObservational studyDisease 2019Long-term effectsSurvivors' qualityClinical protocolsLong haulersOrgan systems
2022
Rationale and design of the SARCoidosis Outcomes in all respiratory Viral Infectious Diseases (SARCOVID) Study
Strykowski R, Patel DC, Neto MR, Hena KM, Gulati M, Maier LA, Patterson K. Rationale and design of the SARCoidosis Outcomes in all respiratory Viral Infectious Diseases (SARCOVID) Study. BMJ Open Respiratory Research 2022, 9: e001254. PMID: 35882424, PMCID: PMC9329732, DOI: 10.1136/bmjresp-2022-001254.Peer-Reviewed Original ResearchConceptsLung functionRespiratory infectionsLocal institutional review board approvalFibrotic pulmonary sarcoidosisInstitutional review board approvalNon-infected patientsInterstitial lung diseaseRespiratory viral illnessReview board approvalViral infectious diseasesImpact of infectionPulmonary sarcoidosisViral illnessClinical courseInfectious eventsStudy entryStudy cohortPoor outcomeProspective studyLung diseaseLong-term impactLife measuresSarcoidosisHigh riskGeneral populationOccupational and environmental exposures in the Genomic Research in Alpha-1 Antitrypsin Deficiency and Sarcoidosis (GRADS) study
Ryan SM, Mroz MM, Herzog EL, Ryu C, Fingerlin TE, Maier LA, Gulati M. Occupational and environmental exposures in the Genomic Research in Alpha-1 Antitrypsin Deficiency and Sarcoidosis (GRADS) study. Respiratory Medicine 2022, 200: 106923. PMID: 35932543, DOI: 10.1016/j.rmed.2022.106923.Peer-Reviewed Original ResearchConceptsAlpha-1 antitrypsin deficiencyCardiac sarcoidosisSarcoidosis StudyAntitrypsin deficiencyRadiation exposureNon-cardiac sarcoidosisNon-chronic diseasesMultiple comparisonsEnvironmental exposure dataExtrapulmonary diseasePulmonary involvementGranulomatous disorderSarcoidosis phenotypesRisk factorsSpecific disease phenotypesStage 0Number of exposuresSarcoidosisExposure assessment toolsSusceptible individualsSpecific exposuresDiseaseEnvironmental exposuresExposure dataIndividual exposureInhibition of type 1 immunity with tofacitinib is associated with marked improvement in longstanding sarcoidosis
Damsky W, Wang A, Kim DJ, Young BD, Singh K, Murphy MJ, Daccache J, Clark A, Ayasun R, Ryu C, McGeary MK, Odell ID, Fazzone-Chettiar R, Pucar D, Homer R, Gulati M, Miller EJ, Bosenberg M, Flavell RA, King B. Inhibition of type 1 immunity with tofacitinib is associated with marked improvement in longstanding sarcoidosis. Nature Communications 2022, 13: 3140. PMID: 35668129, PMCID: PMC9170782, DOI: 10.1038/s41467-022-30615-x.Peer-Reviewed Original ResearchConceptsType 1 immunityInternal organ involvementOrgan involvementT cell-derived IFNIdiopathic inflammatory disorderOpen-label trialMonths of treatmentType 1 cytokinesJanus kinase inhibitorImmunologic changesLongstanding sarcoidosisSarcoidosis activitySarcoidosis symptomsClinical improvementCutaneous sarcoidosisSecondary outcomesComplete responsePrimary outcomeTofacitinib treatmentActivity scoreCytokine mediatorsIL-12IL-15IL-6Inflammatory disordersDisparities 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 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 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 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 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
2021
Transcriptomics of bronchoalveolar lavage cells identifies new molecular endotypes of sarcoidosis
Vukmirovic M, Yan X, Gibson KF, Gulati M, Schupp JC, DeIuliis G, Adams TS, Hu B, Mihaljinec A, Woolard TN, Lynn H, Emeagwali N, Herzog EL, Chen ES, Morris A, Leader JK, Zhang Y, Garcia JGN, Maier LA, Collman RG, Drake WP, Becich MJ, Hochheiser H, Wisniewski SR, Benos PV, Moller DR, Prasse A, Koth LL, Kaminski N. Transcriptomics of bronchoalveolar lavage cells identifies new molecular endotypes of sarcoidosis. European Respiratory Journal 2021, 58: 2002950. PMID: 34083402, PMCID: PMC9759791, DOI: 10.1183/13993003.02950-2020.Peer-Reviewed Original ResearchConceptsWeighted gene co-expression network analysisGene co-expression network analysisCo-expression network analysisGene expression programsGene expression patternsDistinct transcriptional programsImmune response pathwaysIon Torrent ProtonMicroarray expression datasetsExpression programsTranscriptional programsPhenotypic traitsGene modulesResponse pathwaysRNA sequencingMolecular endotypesExpression patternsGene expressionHilar lymphadenopathyOrgan involvementGenomic researchMechanistic targetExpression datasetsT helper type 1T cell immune responses
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 sitesRegistryA Clinic Blueprint for Post-Coronavirus Disease 2019 RECOVERY Learning From the Past, Looking to the Future
Lutchmansingh DD, Knauert MP, Antin-Ozerkis DE, Chupp G, Cohn L, Dela Cruz CS, Ferrante LE, Herzog EL, Koff J, Rochester CL, Ryu C, Singh I, Tickoo M, Winks V, Gulati M, Possick JD. A Clinic Blueprint for Post-Coronavirus Disease 2019 RECOVERY Learning From the Past, Looking to the Future. CHEST Journal 2020, 159: 949-958. PMID: 33159907, PMCID: PMC7641526, DOI: 10.1016/j.chest.2020.10.067.Peer-Reviewed Reviews, Practice Guidelines, Standards, and Consensus StatementsConceptsAcute diseaseRespiratory syndromeSevere acute respiratory syndrome coronavirus 2Acute respiratory syndrome coronavirus 2Post-intensive care syndromePotential long-term complicationsCOVID-19Respiratory syndrome coronavirus 2Middle East respiratory syndromeSevere acute respiratory syndromeCoronavirus disease 2019 (COVID-19) casesPersistent respiratory symptomsLong-term complicationsCOVID-19 survivorsSyndrome coronavirus 2Acute respiratory syndromeCOVID-19 outcomesChronic complicationsPersistent symptomsRespiratory symptomsCoronavirus 2Lung diseaseNonhospitalized individualsClinical programsComplicationsAntifibrotic 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 historyEssential Components of an Interstitial Lung Disease Clinic Results From a Delphi Survey and Patient Focus Group Analysis
Graney B, He C, Marll M, Matson S, Bianchi P, Cosgrove G, Lee J, Collaborators P, Abrencillo R, Bascom R, Scholand M, Bhatt N, Case A, Chaudhary S, Culver D, Danoff S, Desai A, Dilling D, Glazer C, Gulati M, Gupta N, Hamblin M, Hamzeh N, Huie T, Kim H, King C, Kreider M, Lacamera P, Lancaster L, Luckhardt T, Mageto Y, Kottman R, McCormick J, Mehrad B, Menon P, Montesi S, Mooney J, Moore D, Moua T, Nambiar A, Oldham J, Patel D, Paul T, Perez R, Podolanczuk A, Ramaswamy M, Roe D, Saad M, Sandbo N, Schaumberg T, Schmidt S, Shea B, Shifren A, Strek M, Thavarajah K, Todd N, Veeraraghavan S, Weight S, Wolters P, Zibrak J. Essential Components of an Interstitial Lung Disease Clinic Results From a Delphi Survey and Patient Focus Group Analysis. CHEST Journal 2020, 159: 1517-1530. PMID: 33031832, PMCID: PMC7534733, DOI: 10.1016/j.chest.2020.09.256.Peer-Reviewed Original ResearchConceptsInterstitial lung diseaseILD clinicCaregiver focus groupsPhysician expertsInterstitial lung disease clinicPatient-centered medical careManagement of patientsPulmonary Fibrosis FoundationDelphi surveyILD expertsILD patientsDisease clinicMultidisciplinary careLung diseasePatient outcomesFocus groupsClinicPatientsMedical careStudy designRound 1Essential componentThree-roundCareFocus group analysisDiagnosis of Hypersensitivity Pneumonitis in Adults. An Official ATS/JRS/ALAT Clinical Practice Guideline
Raghu G, Remy-Jardin M, Ryerson CJ, Myers JL, Kreuter M, Vasakova M, Bargagli E, Chung JH, Collins BF, Bendstrup E, Chami HA, Chua AT, Corte TJ, Dalphin JC, Danoff SK, Diaz-Mendoza J, Duggal A, Egashira R, Ewing T, Gulati M, Inoue Y, Jenkins AR, Johannson KA, Johkoh T, Tamae-Kakazu M, Kitaichi M, Knight SL, Koschel D, Lederer DJ, Mageto Y, Maier LA, Matiz C, Morell F, Nicholson AG, Patolia S, Pereira CA, Renzoni EA, Salisbury ML, Selman M, Walsh SLF, Wuyts WA, Wilson KC. Diagnosis of Hypersensitivity Pneumonitis in Adults. An Official ATS/JRS/ALAT Clinical Practice Guideline. American Journal Of Respiratory And Critical Care Medicine 2020, 202: e36-e69. PMID: 32706311, PMCID: PMC7397797, DOI: 10.1164/rccm.202005-2032st.Peer-Reviewed Original ResearchConceptsSurgical lung biopsyHypersensitivity pneumonitisLung biopsyGuidelines CommitteeDiagnosis of HPFibrotic hypersensitivity pneumonitisNonfibrotic hypersensitivity pneumonitisTransbronchial lung biopsyTransbronchial lung cryobiopsyInterstitial lung diseaseJapanese Respiratory SocietyBronchoalveolar lavage fluidClinical practice guidelinesAmerican Thoracic SocietyPotential exposureGRADE approachLung cryobiopsyLavage fluidSerum IgGLung diseasePathological featuresThorough historyRespiratory SocietyThoracic SocietyPractice guidelinesAssociations 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 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 dataPlasma 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