2024
Single-Cell Profiling Reveals Immune Aberrations in Progressive Idiopathic Pulmonary Fibrosis.
Unterman A, Zhao A, Neumark N, Schupp J, Ahangari F, Cosme C, Sharma P, Flint J, Stein Y, Ryu C, Ishikawa G, Sumida T, Gomez J, Herazo-Maya J, Dela Cruz C, Herzog E, Kaminski N. Single-Cell Profiling Reveals Immune Aberrations in Progressive Idiopathic Pulmonary Fibrosis. American Journal Of Respiratory And Critical Care Medicine 2024, 210: 484-496. PMID: 38717443, PMCID: PMC11351796, DOI: 10.1164/rccm.202306-0979oc.Peer-Reviewed Original ResearchStable idiopathic pulmonary fibrosisIdiopathic pulmonary fibrosisPeripheral blood mononuclear cellsProgressive idiopathic pulmonary fibrosisPeripheral immune systemT cellsPulmonary fibrosisCohort of IPF patientsAssociated with decreased survivalIdiopathic pulmonary fibrosis patientsPeripheral blood mononuclear cell samplesPeripheral blood cell populationsImmune systemFraction of TregsRegulatory T cellsBlood mononuclear cellsBlood cell populationsFlow cytometry analysisImmune aberrationsIPF patientsTregsMononuclear cellsSingle-cell RNA sequencingLung homogenatesMonocyte chemoattractant
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
S2K 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