2024
129Xe magnetic resonance imaging for monitoring lung function and microstructure in patients on treatment for progressive fibrosing interstitial lung disease
Kern A, Voskrebenzev A, Klimeš F, Pink I, Schupp J, Seeliger B, Konietzke M, Peter D, Risse F, Welte T, Wacker F, Hohlfeld J, Shin H, Prasse A, Vogel-Claussen J. 129Xe magnetic resonance imaging for monitoring lung function and microstructure in patients on treatment for progressive fibrosing interstitial lung disease. 2024, oa5465. DOI: 10.1183/13993003.congress-2024.oa5465.Peer-Reviewed Original ResearchSingle Cell Analysis Reveals Novel Immune Perturbations in Fibrotic Hypersensitivity Pneumonitis.
Zhao A, Unterman A, Abu Hussein N, Sharma P, Nekola F, Flint J, Yan X, Adams T, Justet A, Sumida T, Zhao J, Schupp J, Raredon M, Ahangari F, Deluliis G, Zhang Y, Buendia-Roldan I, Adegunsoye A, Sperling A, Prasse A, Ryu C, Herzog E, Selman M, Pardo A, Kaminski N. Single Cell Analysis Reveals Novel Immune Perturbations in Fibrotic Hypersensitivity Pneumonitis. American Journal Of Respiratory And Critical Care Medicine 2024 PMID: 38924775, DOI: 10.1164/rccm.202401-0078oc.Peer-Reviewed Original ResearchFibrotic hypersensitivity pneumonitisIdiopathic pulmonary fibrosisPeripheral blood mononuclear cellsBronchoalveolar lavage cellsBlood mononuclear cellsClassical monocytesHypersensitivity pneumonitisPulmonary fibrosisT cellsImmune perturbationsLavage cellsMononuclear cellsCD8+ T cellsCytotoxic T cellsInterstitial lung diseaseHypersensitivity pneumonitis patientsCytotoxic CD4Immune aberrationsPneumonic patientsPneumonitisLung diseaseHealthy controlsImmune mechanismsPatient cellsSingle-cell transcriptomics
2023
Multilamellated Basement Membranes in the Capillary Network of Alveolar Capillary Dysplasia
Kamp J, Neubert L, Schupp J, Braubach P, Wrede C, Laenger F, Salditt T, Reichmann J, Welte T, Ruhparwar A, Ius F, Schwerk N, Bergmann A, von Hardenberg S, Griese M, Rapp C, Olsson K, Fuge J, Park D, Hoeper M, Jonigk D, Knudsen L, Kuehnel M. Multilamellated Basement Membranes in the Capillary Network of Alveolar Capillary Dysplasia. American Journal Of Pathology 2023, 194: 180-194. PMID: 38029923, DOI: 10.1016/j.ajpath.2023.10.012.Peer-Reviewed Original Research
2022
Lung Microenvironments and Disease Progression in Fibrotic Hypersensitivity Pneumonitis.
De Sadeleer LJ, McDonough JE, Schupp JC, Yan X, Vanstapel A, Van Herck A, Everaerts S, Geudens V, Sacreas A, Goos T, Aelbrecht C, Nawrot TS, Martens DS, Schols D, Claes S, Verschakelen JA, Verbeken EK, Ackermann M, Decottignies A, Mahieu M, Hackett TL, Hogg JC, Vanaudenaerde BM, Verleden SE, Kaminski N, Wuyts WA. Lung Microenvironments and Disease Progression in Fibrotic Hypersensitivity Pneumonitis. American Journal Of Respiratory And Critical Care Medicine 2022, 205: 60-74. PMID: 34724391, PMCID: PMC8865586, DOI: 10.1164/rccm.202103-0569oc.Peer-Reviewed Original ResearchConceptsFibrotic hypersensitivity pneumonitisIdiopathic pulmonary fibrosisHypersensitivity pneumonitisLung zonesMolecular traitsUnused donor lungsInterstitial lung diseaseLocal disease extentProgression of fibrosisSevere fibrosis groupGene co-expression network analysisCo-expression network analysisExplant lungsDonor lungsLung involvementEndothelial functionLung findingsDisease extentPulmonary fibrosisLung diseaseFibrosis groupLung microenvironmentClinical behaviorDisease progressionBAL samples
2021
Bronchoalveolar Lavage Fluid Reflects a TH1-CD21low B-Cell Interaction in CVID-Related Interstitial Lung Disease
Friedmann D, Unger S, Keller B, Rakhmanov M, Goldacker S, Zissel G, Frye B, Schupp J, Prasse A, Warnatz K. Bronchoalveolar Lavage Fluid Reflects a TH1-CD21low B-Cell Interaction in CVID-Related Interstitial Lung Disease. Frontiers In Immunology 2021, 11: 616832. PMID: 33613543, PMCID: PMC7892466, DOI: 10.3389/fimmu.2020.616832.Peer-Reviewed Original ResearchConceptsInterstitial lung diseaseCommon variable immunodeficiencyBAL fluidB cellsLung diseaseT cellsImmune dysregulationBronchoalveolar lavageHealthy donorsClass-switched memory B cellsLymphocytic interstitial lung diseaseProgressive interstitial lung diseaseLevels of BAFFLike cell subsetSystemic immune dysregulationRegulatory T cellsVideo-assisted thoracoscopyBronchoalveolar lavage fluidT cell populationsPotential immune mechanismsLevels of APRILMemory B cellsB cell interactionsFlow cytometric profilingPotential treatment targetS2K Guideline for Diagnosis of Idiopathic Pulmonary Fibrosis
Behr J, Günther A, Bonella F, Dinkel J, Fink L, Geiser T, Geissler K, Gläser S, Handzhiev S, Jonigk D, Koschel D, Kreuter M, Leuschner G, Markart P, Prasse A, Schönfeld N, Schupp J, Sitter H, Müller-Quernheim J, Costabel U. S2K Guideline for Diagnosis of Idiopathic Pulmonary Fibrosis. Respiration 2021, 100: 238-271. PMID: 33486500, DOI: 10.1159/000512315.Peer-Reviewed Reviews, Practice Guidelines, Standards, and Consensus StatementsConceptsIdiopathic pulmonary fibrosisPulmonary fibrosisDiagnosis of IPFInternational IPF guidelinesSurgical lung biopsyDiagnosis of exclusionTransbronchial lung cryobiopsyInterstitial lung diseaseIPF patientsTypical clinical contextLung biopsyLung cryobiopsyBronchoalveolar lavageSerologic testingBronchoscopic diagnosisLung diseaseDiagnostic workupIPF guidelinesMultidisciplinary discussionStandardized questionnaireFatal diseaseUpdate 2018DiagnosisClinical contextGolden standard
2020
S2K-Leitlinie zur Diagnostik der idiopathischen Lungenfibrose
Behr J, Günther A, Bonella F, Dinkel J, Fink L, Geiser T, Geißler K, Gläser S, Handzhhiev S, Jonigk D, Koschel D, Kreuter M, Leuschner G, Markart P, Prasse A, Schönfeld N, Schupp J, Sitter H, Müller-Quernheim J, Costabel U. S2K-Leitlinie zur Diagnostik der idiopathischen Lungenfibrose. Pneumologie 2020, 74: 263-293. PMID: 32227328, DOI: 10.1055/a-1120-3531.Peer-Reviewed Reviews, Practice Guidelines, Standards, and Consensus StatementsConceptsIdiopathic pulmonary fibrosisIPF patientsDiagnosis of IPFInternational IPF guidelinesSurgical lung biopsyDiagnosis of exclusionTransbronchial lung cryobiopsyInterstitial lung diseaseTypical clinical settingLung biopsyLung cryobiopsyBronchoalveolar lavagePulmonary fibrosisSerologic testingBronchoscopic diagnosisLung diseaseIPF guidelinesAntifibrotic drugsMultidisciplinary discussionFatal diseaseUpdate 2018Standardised questionnaireClinical settingPatientsDiagnosis
2016
Usefulness of Cyclophosphamide Pulse Therapy in Interstitial Lung Diseases
Schupp J, Köhler T, Müller-Quernheim J. Usefulness of Cyclophosphamide Pulse Therapy in Interstitial Lung Diseases. Respiration 2016, 91: 296-301. PMID: 27082957, DOI: 10.1159/000445031.Peer-Reviewed Original ResearchConceptsNon-specific interstitial pneumoniaInterstitial lung diseaseLymphocytic interstitial pneumoniaCyclophosphamide pulse therapyLung functionPulse therapyInterstitial pneumoniaLung diseaseRheumatoid arthritis-associated interstitial lung diseaseIdiopathic non-specific interstitial pneumoniaFibrotic interstitial lung diseaseProgressive lung function declineBetter survival outcomesLung function declineIdiopathic pulmonary fibrosisBronchoalveolar lavage cytologyGroup of disordersILD patientsMost patientsSystemic sclerosisTotal cohortFunction declineLavage cytologyPulmonary fibrosisCyclophosphamide treatment