2023
Increased expression of CXCL6 in secretory cells drives fibroblast collagen synthesis and is associated with increased mortality in idiopathic pulmonary fibrosis.
Bahudhanapati H, Tan J, Apel R, Seeliger B, Schupp J, Li X, Sullivan D, Sembrat J, Rojas M, Tabib T, Valenzi E, Lafyatis R, Mitash N, Hernandez Pineda R, Jawale C, Peroumal D, Biswas P, Tedrow J, Adams T, Kaminski N, Wuyts W, McDyer J, Gibson K, Alder J, Königshoff M, Zhang Y, Nouraie M, Prasse A, Kass D. Increased expression of CXCL6 in secretory cells drives fibroblast collagen synthesis and is associated with increased mortality in idiopathic pulmonary fibrosis. European Respiratory Journal 2023, 63: 2300088. PMID: 37918852, DOI: 10.1183/13993003.00088-2023.Peer-Reviewed Original ResearchConceptsIdiopathic pulmonary fibrosisAirway epithelial cellsBronchoalveolar lavagePulmonary fibrosisEpithelial cellsCollagen synthesisPathogenesis of IPFCohort of patientsIPF lung fibroblastsEffects of chemokinesAir-liquid interface culturesExpression of CXCL6Collagen I levelsIPF mortalityIPF patientsChemokine levelsIPF fibroblastsPoor survivalDistal lungI levelsWhole lungAnimal modelsEctopic localisationPatientsSingle-cell RNA sequencing
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
Gene coexpression networks reveal novel molecular endotypes in alpha-1 antitrypsin deficiency
Chu JH, Zang W, Vukmirovic M, Yan X, Adams T, DeIuliis G, Hu B, Mihaljinec A, Schupp JC, Becich MJ, Hochheiser H, Gibson KF, Chen ES, Morris A, Leader JK, Wisniewski SR, Zhang Y, Sciurba FC, Collman RG, Sandhaus R, Herzog EL, Patterson KC, Sauler M, Strange C, Kaminski N. Gene coexpression networks reveal novel molecular endotypes in alpha-1 antitrypsin deficiency. Thorax 2020, 76: 134-143. PMID: 33303696, PMCID: PMC10794043, DOI: 10.1136/thoraxjnl-2019-214301.Peer-Reviewed Original ResearchConceptsWeighted gene co-expression network analysisAlpha-1 antitrypsin deficiencyGene modulesGene co-expression network analysisDifferential gene expression analysisCo-expression network analysisPeripheral blood mononuclear cellsGene expression patternsPBMC gene expression patternsGene coexpression networksAATD individualsGene expression profilesGene expression analysisBronchoalveolar lavageAugmentation therapyClinical variablesAntitrypsin deficiencyGene expression assaysRNA-seqCoexpression networkGene validationExpression analysisExpression assaysWGCNA modulesExpression patternsS2K-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
2013
Lung Collagens Perpetuate Pulmonary Fibrosis via CD204 and M2 Macrophage Activation
Stahl M, Schupp J, Jäger B, Schmid M, Zissel G, Müller-Quernheim J, Prasse A. Lung Collagens Perpetuate Pulmonary Fibrosis via CD204 and M2 Macrophage Activation. PLOS ONE 2013, 8: e81382. PMID: 24278429, PMCID: PMC3835428, DOI: 10.1371/journal.pone.0081382.Peer-Reviewed Original ResearchConceptsIdiopathic pulmonary fibrosisPulmonary fibrosisAlveolar macrophagesCollagen type IHealthy donorsInterleukin-1 receptor antagonistType IM2 macrophage activationExpression of CCL2Lower respiratory tractIL-1ra productionAbundant collagen productionInnate immune responsePhospho-Akt expressionType I effectsExpression of CD204Collagen breakdown productsBronchoalveolar lavageReceptor antagonistCD204 expressionRespiratory tractLung collagenImmune responseM2 typeMacrophage activation