2024
Bexotegrast in Patients with Idiopathic Pulmonary Fibrosis: The INTEGRIS-IPF Clinical Trial
Lancaster L, Cottin V, Ramaswamy M, Wuyts W, Jenkins R, Scholand M, Kreuter M, Valenzuela C, Ryerson C, Goldin J, Kim G, Jurek M, Decaris M, Clark A, Turner S, Barnes C, Achneck H, Cosgrove G, Lefebvre É, Flaherty K, Baratz D, Ettinger N, Layish D, Epstein M, Veeraraghavan S, Golden J, Ramaswamy M, Bascom R, Lancaster L, Scholand M, Case A, Zaman T, Betensley A, Antin-Ozerkis D, Montessi S, Fernandez E, Boente R, Sager J, Hunninghake G, Gibson K, Srour N, Dhar A, Wuyts W, Wielders P, Veltkamp M, Mostard R, Janssen R, Noordegraaf A, Glaspole I, Corte T, Beckert L, Brockway B, Veale A, Richeldi L, Harari S. Bexotegrast in Patients with Idiopathic Pulmonary Fibrosis: The INTEGRIS-IPF Clinical Trial. American Journal Of Respiratory And Critical Care Medicine 2024, 210: 424-434. PMID: 38843105, PMCID: PMC11351797, DOI: 10.1164/rccm.202403-0636oc.Peer-Reviewed Original ResearchMeSH KeywordsAgedDose-Response Relationship, DrugDouble-Blind MethodFemaleHumansIdiopathic Pulmonary FibrosisIndolesMaleMiddle AgedPyridonesTreatment OutcomeConceptsTreatment-emergent adverse eventsQuantitative lung fibrosisIdiopathic pulmonary fibrosisPulmonary fibrosisRates of treatment-emergent adverse eventsIncidence of treatment-emergent adverse eventsClinical trialsTreatment of idiopathic pulmonary fibrosisExploratory efficacy endpointsImpaired quality of lifeFibrosis-related biomarkersDose cohortsBackground therapyPlacebo groupTolerability profileOnce-dailyEfficacy endpointPrimary endpointProgressive diseaseExertional dyspneaFVC declineFibrosis biomarkersAdverse eventsIPF therapyLung fibrosis
2023
Ziritaxestat, a Novel Autotaxin Inhibitor, and Lung Function in Idiopathic Pulmonary Fibrosis
Maher T, Ford P, Brown K, Costabel U, Cottin V, Danoff S, Groenveld I, Helmer E, Jenkins R, Milner J, Molenberghs G, Penninckx B, Randall M, Van Den Blink B, Fieuw A, Vandenrijn C, Rocak S, Seghers I, Shao L, Taneja A, Jentsch G, Watkins T, Wuyts W, Kreuter M, Verbruggen N, Prasad N, Wijsenbeek M, Chambers D, Chia M, Corte T, Glaspole I, Goh N, Holmes M, Malouf M, Thien F, Veitch E, Bondue B, Dahlqvist C, Froidure A, Slabbynck H, Wuyts W, Cartagena Salinas C, Feijoó Seoane R, Martínez V, Maturana R, Pavie Gallegos J, Rosenblut A, Silva R, Undurraga Pereira A, Doubkova M, Pauk N, Plackova M, Sterclova M, Bendstrup E, Shaker S, Titlestad I, Budweiser S, Grohé C, Koschel D, Kreuter M, Prasse A, Weber M, Wirtz H, Antoniou K, Daniil Z, Gaga M, Papakosta D, Izumi S, Okamoto M, Guerreros Benavides A, Iberico Barrera C, Peña Villalobos A, Campo Ezquibela A, Cifrian Martinez J, Fernandez Fabrellas E, Leiro V, Molina-Molina M, Nieto Barbero A, Sellares Torres J, Valenzuela C, Cheng S, Kuo P, Lee K, Sheu C, Gunen H, Mogulkoc Bishop N, Nayci S, Adamali H, Bianchi S, Chaudhuri N, Gibbons M, Hart S, Molyneaux P, Parfrey H, Saini G, Spencer L, Wiscombe S, Antin-Ozerkis D, Bascom R, Belperio J, Britt E, Fitzgerald J, Gomez Manjarres D, Gotfried M, Gupta N, Hotchkin D, Kaye M, Kreider M, Kureishy S, Lacamera P, Lancaster L, Lasky J, Lorch D, Mannem H, Morrow L, Moua T, Nambiar A, Raghu G, Raj R, Ramaswamy M, Reddy R, Russell T, Scholand M, Shea B, Suliman S, Swigris J, Thavarajah K, Tolle L, Tomic R, Warshoff N, Wesselius L, Yung G, Bergna M, De Salvo M, Fernandez Acquier M, Rodriguez A, Saez Scherbovsky P, Assayag D, Dhar A, Khalil N, Morisset J, Provencher S, Ryerson C, Shapera S, Bourdin A, Crestani B, Lebargy F, Reynaud-Gaubert M, Bonella F, Claussen M, Hammerl P, Karagiannidis C, Keller C, Randerath W, Stubbe B, Csánky E, Medgyasszay B, Muller V, Adir Y, Bar-Shai A, Berkman N, Fink G, Kramer M, Shitrit D, Bargagli E, Gasparini S, Harari S, Ravaglia C, Richeldi L, Vancheri C, Ebina M, Fujita M, Ichikado K, Inoue Y, Ishikawa N, Kato M, Kawamura T, Kondoh Y, Nishioka Y, Ogura T, Owan I, Saito T, Sakamoto N, Sakamoto K, Shirai M, Suda T, Tomii K, Chung M, Jeong S, Park C, Park J, Song J, Uh S, Chavarria Martinez U, Montano Gonzalez E, Ramirez A, Selman Lama M, Bresser P, Kramer H, Mostard R, Nossent E, Veltkamp M, Wijsenbeek M, Beckert L, Chang C, Veale A, Wilsher M, Bednarek M, Gasior G, Jasieniak-Pinis G, Jassem E, Mroz R, Piotrowski W, Abdullah I, Ambaram A, Irusen E, Van der Linden M, van Zyl-Smit R, Williams P, Allen J, Averill F, Belloli E, Brown A, Case A, Chaudhary S, Criner G, DeBoer K, Dilling D, Dorf J, Enelow R, Ettinger N, Feldman J, Gibson K, Golden J, Hamblin M, Hunninghake G, Karunakara R, Kim H, Luckhardt T, Menon P, Morrison L, Oldham J, Patel N, Schmidt S, Strek M, Summer R, Sussman R, Tita J, Veeraraghavan S, Whelan T, Zibrak J. Ziritaxestat, a Novel Autotaxin Inhibitor, and Lung Function in Idiopathic Pulmonary Fibrosis. JAMA 2023, 329: 1567-1578. PMID: 37159034, PMCID: PMC10170340, DOI: 10.1001/jama.2023.5355.Peer-Reviewed Original ResearchMeSH KeywordsAdministration, OralAgedClinical Trials, Phase III as TopicFemaleHumansIdiopathic Pulmonary FibrosisLungMaleMiddle AgedMulticenter Studies as TopicPhosphodiesterase InhibitorsQuality of LifeRandomized Controlled Trials as TopicRespiratory Physiological PhenomenaRespiratory System AgentsTreatment OutcomeConceptsIdiopathic pulmonary fibrosisKey secondary outcomesFVC declineSecondary outcomesPulmonary fibrosisCare treatmentSt George's Respiratory Questionnaire (SGRQ) total scoreRespiratory-related hospitalizationsSafety Monitoring CommitteeQuestionnaire total scoreNovel autotaxin inhibitorAnnual rateCause mortalityWeek 52Lung functionPrimary outcomeClinical outcomesVital capacityStudy terminationClinical trialsDisease progressionPlaceboMAIN OUTCOMEPatientsAutotaxin inhibitors
2022
Long-term evaluation of the safety and efficacy of recombinant human pentraxin-2 (rhPTX-2) in patients with idiopathic pulmonary fibrosis (IPF): an open-label extension study
Raghu G, Hamblin MJ, Brown AW, Golden JA, Ho LA, Wijsenbeek MS, Vasakova M, Pesci A, Antin-Ozerkis DE, Meyer KC, Kreuter M, Burgess T, Kamath N, Donaldson F, Richeldi L. Long-term evaluation of the safety and efficacy of recombinant human pentraxin-2 (rhPTX-2) in patients with idiopathic pulmonary fibrosis (IPF): an open-label extension study. Respiratory Research 2022, 23: 129. PMID: 35597980, PMCID: PMC9123757, DOI: 10.1186/s12931-022-02047-0.Peer-Reviewed Original ResearchConceptsOpen-label extensionIdiopathic pulmonary fibrosisWeek 128PRM-151Serious TEAEsPulmonary fibrosisPentraxin 2Efficacy dataRandomized periodTreatment-emergent adverse event profilesOpen-label extension studyAcute respiratory failureAdverse event profileNew safety signalsPatient-reported outcomesLimited efficacy dataLong-term safetyEudraCT 2014Lung transplantationRespiratory failureWalk distanceLong-term evaluationEvent profileVital capacityIntravenous infusion
2020
Nintedanib in patients with progressive fibrosing interstitial lung diseases—subgroup analyses by interstitial lung disease diagnosis in the INBUILD trial: a randomised, double-blind, placebo-controlled, parallel-group trial
Wells A, Flaherty K, Brown K, Inoue Y, Devaraj A, Richeldi L, Moua T, Crestani B, Wuyts W, Stowasser S, Quaresma M, Goeldner R, Schlenker-Herceg R, Kolb M, investigators I, Abe S, Aburto M, Acosta O, Andrews C, Antin-Ozerkis D, Arce G, Arias M, Avdeev S, Barczyk A, Bascom R, Bazdyrev E, Beirne P, Belloli E, Bergna M, Bergot E, Bhatt N, Blaas S, Bondue B, Bonella F, Britt E, Buch K, Burk J, Cai H, Cantin A, Villegas D, Cazaux A, Cerri S, Chaaban S, Chaudhuri N, Cottin V, Crestani B, Criner G, Dahlqvist C, Danoff S, D'Amico J, Dilling D, Elias P, Ettinger N, Falk J, Pérez E, Gamez-Dubuis A, Giessel G, Gifford A, Glassberg M, Glazer C, Golden J, Carrera L, Guiot J, Hallowell R, Hayashi H, Hetzel J, Hirani N, Homik L, Hope-Gill B, Hotchkin D, Ichikado K, Ilkovich M, Inoue Y, Izumi S, Jassem E, Jones L, Jouneau S, Kaner R, Kang J, Kawamura T, Kessler R, Kim Y, Kishi K, Kitamura H, Kolb M, Kondoh Y, Kono C, Koschel D, Kreuter M, Kulkarni T, Kus J, Lebargy F, Jiménez A, Luo Q, Mageto Y, Maher T, Makino S, Marchand-Adam S, Marquette C, Martinez R, Martínez M, Rozas R, Miyazaki Y, Moiseev S, Molina-Molina M, Morrison L, Morrow L, Moua T, Nambiar A, Nishioka Y, Nunes H, Okamoto M, Oldham J, Otaola M, Padilla M, Park J, Patel N, Pesci A, Piotrowski W, Pitts L, Poonyagariyagorn H, Prasse A, Quadrelli S, Randerath W, Refini R, Reynaud-Gaubert M, Riviere F, Portal J, Rosas I, Rossman M, Safdar Z, Saito T, Sakamoto N, Fénero M, Sauleda J, Schmidt S, Scholand M, Schwartz M, Shapera S, Shlobin O, Sigal B, Orellana A, Skowasch D, Song J, Stieglitz S, Stone H, Strek M, Suda T, Sugiura H, Takahashi H, Takaya H, Takeuchi T, Thavarajah K, Tolle L, Tomassetti S, Tomii K, Valenzuela C, Vancheri C, Varone F, Veeraraghavan S, Villar A, Weigt S, Wemeau L, Wuyts W, Xu Z, Yakusevich V, Yamada Y, Yamauchi H, Ziora D. Nintedanib in patients with progressive fibrosing interstitial lung diseases—subgroup analyses by interstitial lung disease diagnosis in the INBUILD trial: a randomised, double-blind, placebo-controlled, parallel-group trial. The Lancet Respiratory Medicine 2020, 8: 453-460. PMID: 32145830, DOI: 10.1016/s2213-2600(20)30036-9.Peer-Reviewed Original ResearchMeSH KeywordsAgedDiarrheaDisease ProgressionDouble-Blind MethodFemaleHumansIdiopathic Pulmonary FibrosisIndolesMaleMiddle AgedNauseaProtein Kinase InhibitorsVital CapacityConceptsInterstitial lung diseaseIdiopathic pulmonary fibrosisIdiopathic non-specific interstitial pneumoniaAutoimmune interstitial lung diseaseUnclassifiable idiopathic interstitial pneumoniaNon-specific interstitial pneumoniaDose of nintedanibParallel-group trialIdiopathic interstitial pneumoniaEffect of nintedanibINBUILD trialInterstitial pneumoniaILD diagnosisHigh-resolution CTILD progressionFVC declineHypersensitivity pneumonitisProgressive fibrosing interstitial lung diseasesOverall populationInterstitial lung disease diagnosisChest imaging featuresProtocol-defined criteriaSafety of nintedanibChronic hypersensitivity pneumonitisDisease subgroup analyses
2019
Update in Pulmonary Fibrosis 2018
Sgalla G, Kulkarni T, Antin-Ozerkis D, Thannickal VJ, Richeldi L. Update in Pulmonary Fibrosis 2018. American Journal Of Respiratory And Critical Care Medicine 2019, 200: 292-300. PMID: 31022351, DOI: 10.1164/rccm.201903-0542up.Peer-Reviewed Original ResearchHumansIdiopathic Pulmonary FibrosisLong-term treatment with recombinant human pentraxin 2 protein in patients with idiopathic pulmonary fibrosis: an open-label extension study
Raghu G, van den Blink B, Hamblin MJ, Brown AW, Golden JA, Ho LA, Wijsenbeek MS, Vasakova M, Pesci A, Antin-Ozerkis DE, Meyer KC, Kreuter M, Moran D, Santin-Janin H, Aubin F, Mulder GJ, Gupta R, Richeldi L. Long-term treatment with recombinant human pentraxin 2 protein in patients with idiopathic pulmonary fibrosis: an open-label extension study. The Lancet Respiratory Medicine 2019, 7: 657-664. PMID: 31122893, DOI: 10.1016/s2213-2600(19)30172-9.Peer-Reviewed Original ResearchMeSH KeywordsAgedCross-Over StudiesDouble-Blind MethodFemaleHomeodomain ProteinsHumansIdiopathic Pulmonary FibrosisLong-Term CareMaleRecombinant ProteinsSerum Amyloid P-ComponentTreatment OutcomeVital CapacityConceptsOpen-label extension studyIdiopathic pulmonary fibrosisPRM-151Adverse eventsLong-term treatmentWalking distanceExtension studyIPF exacerbationsWeek 52Week 76Pulmonary fibrosisIPF progressionDouble-blind treatment periodLife-threatening adverse eventsDouble-blind periodExploratory efficacy analysesSerious adverse eventsSevere adverse eventsMin intravenous infusionLong-term safetyRandom intercept mixed modelChest painPlacebo groupMore patientsEfficacy analysisSerious adverse events in patients with idiopathic pulmonary fibrosis in the placebo arms of 6 clinical trials
Wuyts W, Antin-Ozerkis D, Huggins JT, LaCamera PP, Spagnolo P, Vašáková M, Wijsenbeek MS, Polman B, Kirchgaessler KU, Scholand MB. Serious adverse events in patients with idiopathic pulmonary fibrosis in the placebo arms of 6 clinical trials. Respiratory Medicine 2019, 150: 120-125. PMID: 30961937, DOI: 10.1016/j.rmed.2019.02.021.Peer-Reviewed Original ResearchConceptsSerious adverse eventsIdiopathic pulmonary fibrosisAdverse eventsPulmonary fibrosisClinical trialsCardiac disordersFatal interstitial lung diseaseInterstitial lung diseaseRespiratory failureCommon comorbiditiesPlacebo armLung functionFatal outcomeLung diseasePooled analysisMyocardial infarctionUnpredictable courseDisease progressionFatal infectionSafety dataPatientsPlaceboFibrosisInfectionTrials
2018
Effect of Recombinant Human Pentraxin 2 vs Placebo on Change in Forced Vital Capacity in Patients With Idiopathic Pulmonary Fibrosis: A Randomized Clinical Trial
Raghu G, van den Blink B, Hamblin MJ, Brown AW, Golden JA, Ho LA, Wijsenbeek MS, Vasakova M, Pesci A, Antin-Ozerkis DE, Meyer KC, Kreuter M, Santin-Janin H, Mulder GJ, Bartholmai B, Gupta R, Richeldi L. Effect of Recombinant Human Pentraxin 2 vs Placebo on Change in Forced Vital Capacity in Patients With Idiopathic Pulmonary Fibrosis: A Randomized Clinical Trial. JAMA 2018, 319: 2299-2307. PMID: 29800034, PMCID: PMC6134440, DOI: 10.1001/jama.2018.6129.Peer-Reviewed Original ResearchMeSH KeywordsAgedDouble-Blind MethodFemaleHomeodomain ProteinsHumansIdiopathic Pulmonary FibrosisLeast-Squares AnalysisMaleMiddle AgedRecombinant ProteinsSerum Amyloid P-ComponentVital CapacityWalk TestConceptsIdiopathic pulmonary fibrosisPentraxin 2Placebo groupPulmonary fibrosisWeek 28FVC percentageWalk distanceLung volumeProgressive fibrotic lung diseaseEnd pointVital capacity percentageCommon adverse eventsPlacebo-controlled trialPrimary end pointSecondary end pointsFibrotic lung diseaseForced Vital CapacityTotal lung volumeSignificant treatment differencesEligible patientsMeasurements of DLCOStudy drugAdverse eventsLung functionPoor prognosis
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
2013
Connective tissue disease related interstitial lung diseases and idiopathic pulmonary fibrosis: provisional core sets of domains and instruments for use in clinical trials
Saketkoo L, Mittoo S, Huscher D, Khanna D, Dellaripa P, Distler O, Flaherty K, Frankel S, Oddis C, Denton C, Fischer A, Kowal-Bielecka O, LeSage D, Merkel P, Phillips K, Pittrow D, Swigris J, Antoniou K, Baughman R, Castelino F, Christmann R, Christopher-Stine L, Collard H, Cottin V, Danoff S, Highland K, Hummers L, Shah A, Kim D, Lynch D, Miller F, Proudman S, Richeldi L, Ryu J, Sandorfi N, Sarver C, Wells A, Strand V, Matteson E, Brown K, Seibold J, Aggarwal* R, Ainslie G, Alkassab F, Allanore Y, Descartes P, Anderson M, Andonopoulos A, Antin-Ozerkis D, Arrobas A, Ascherman* D, Assassi S, Baron M, Bathon* J, Behr J, Beretta L, Bingham C, Binnie M, Birring S, Boin F, Bongartz* T, Bourdin A, Bouros D, Brasington R, Bresser P, Buch M, Burge P, Carmona L, Carreira P, Carvalho C, Catoggio L, Chan K, Chapman J, Chatterjee S, Chua* F, Chung L, Conron M, Corte T, Cosgrove G, Costabel U, Cox G, Crestani B, Crofford L, Csuka M, Curbelo P, László C, Daniil Z, D'Arsigny C, Davis G, de Andrade J, De Vuyst P, Dempsey O, Derk C, Distler J, Dixon* W, Downey G, Doyle M, Drent M, Durairaj L, Emery P, Espinoza L, Farge D, Fathi M, Fell C, Fessler B, Fitzgerald J, Fox G, Foeldvari I, Frech T, Freitas S, Furst* D, Gabrielli A, García-Vicuña R, Georgiev O, Gerbino A, Gillisen A, Gladman D, Glassberg M, Gochuico B, Gogali A, Goh* N, Goldberg A, Goldberg H, Gourley* M, Griffing L, Grutters J, Gunnarsson R, Hachulla E, Hall F, Harari S, Herrick A, Herzog E, Hesselstrand R, Hirani N, Hodgson U, Hollingsworth H, Homer R, Hoyles R, Hsu V, Hubbard R, Hunzelmann N, Isasi M, Isasi E, Jimenez J, Johnson S, Jones C, Kahaleh B, Kairalla R, Kalluri M, Kalra S, Kaner R, Kinder B, Klingsberg R, Kokosi M, Kolb M, Kur-Zalewska J, Kuwana* M, Lake F, Lally E, Lasky J, Laurindo I, Able L, Lee P, Leonard C, Lien D, Limper A, Liossis S, Lohr K, Loyd J, Lundberg* I, Mageto Y, Maher T, Mahmud T, Manganas H, Marie I, Marras T, Martinez J, Martinez F, Mathieu A, Matucci-Cerinic* M, Mayes* M, McKown K, Medsger T, Meehan R, Cristina M, Meyer K, Millar A, Moğulkoç N, Molitor J, Morais A, Mouthon P, Müller V, Müller-Quernheim J, Nadashkevich O, Nador R, Nash P, Nathan S, Navarro C, Neves S, Noth I, Nunes H, Olson A, Opitz C, Padilla M, Pappas D, Parfrey H, Pego-Reigosa J, Pereira C, Perez R, Pope* J, Porter J, Renzoni E, Riemekasten G, Riley D, Rischmueller M, Rodriguez-Reyna T, Rojas-Serrano, Romam J, Rosen G, Rossman M, Rothfield N, Sahn S, Sanduzzi A, Scholand M, Selman M, Senécal J, Seo P, Silver* R, Solomon J, Steen* V, Stevens W, Strange C, Sussman R, Sutton E, Sweiss N, Tornling G, Tzelepis G, Undurraga A, Vacca A, Vancheri C, Varga J, Veale D, Volkov S, Walker U, Wencel M, Wesselius L, Wickremasinghe M, Wilcox P, Wilsher M, Wollheim F, Wuyts W, Yung G, Zanon P, Zappala C, Groshong S, Leslie K, Myers J, Padera R, Desai S, Goldin J, Kazerooni E, Klein J, Lynch D, Keen K. Connective tissue disease related interstitial lung diseases and idiopathic pulmonary fibrosis: provisional core sets of domains and instruments for use in clinical trials. Thorax 2013, 69: 436. PMID: 24368713, PMCID: PMC3995282, DOI: 10.1136/thoraxjnl-2013-204202.Peer-Reviewed Original ResearchConceptsIdiopathic pulmonary fibrosisConnective tissue diseaseInterstitial lung diseaseCTD-ILDPulmonary fibrosisTissue diseaseLung diseaseClinical trialsOutcome measuresRelated interstitial lung diseaseNominal group panelHealth-related qualityPatient focus groupsMulticentre clinical trialAppropriate outcome measuresClinical trial designILD expertsDisease activityMulticentre RCTsTreatment responseLung physiologyTrial designConsensus methodologyIdentification of outcomesLung imaging