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2. Midregional Proatrial Natriuretic Peptide (MRproANP) is associated with vertebral fractures and low bone density in patients with chronic obstructive pulmonary disease (COPD)

Author

Trudzinski, Franziska C., Jörres, Rudolf A., Alter, Peter, Watz, Henrik, Vogelmeier, Claus F., Kauczor, Hans-Ulrich, Thangamani, Subasini, Debic, Manuel, Welte, Tobias, Behr, Jürgen, Kahnert, Kathrin, Bals, Robert, Herr, Christian, Heußel, Claus Peter, Biederer, Jürgen, von Stackelberg, Oyunbileg, Fähndrich, Sebastian, Wouters, Emiel F. M., Waschki, Benjamin, Rabe, Klaus F., Herth, Felix J. F., and Palm, Viktoria

Published
2024
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4. Disease trajectories in interstitial lung diseases – data from the EXCITING-ILD registry

Author

Buschulte, Katharina, Kabitz, Hans-Joachim, Hagmeyer, Lars, Hammerl, Peter, Esselmann, Albert, Wiederhold, Conrad, Skowasch, Dirk, Stolpe, Christoph, Joest, Marcus, Veitshans, Stefan, Höffgen, Marc, Maqhuzu, Phillen, Schwarzkopf, Larissa, Hellmann, Andreas, Pfeifer, Michael, Behr, Jürgen, Karpavicius, Rainer, Günther, Andreas, Polke, Markus, Höger, Philipp, Somogyi, Vivien, Lederer, Christoph, Markart, Philipp, and Kreuter, Michael

Published
2024
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6. Hospitalisation patterns in interstitial lung diseases: data from the EXCITING-ILD registry

Author

Buschulte, Katharina, Kabitz, Hans-Joachim, Hagmeyer, Lars, Hammerl, Peter, Esselmann, Albert, Wiederhold, Conrad, Skowasch, Dirk, Stolpe, Christoph, Joest, Marcus, Veitshans, Stefan, Höffgen, Marc, Maqhuzu, Phillen, Schwarzkopf, Larissa, Hellmann, Andreas, Pfeifer, Michael, Behr, Jürgen, Karpavicius, Rainer, Günther, Andreas, Polke, Markus, Höger, Philipp, Somogyi, Vivien, Lederer, Christoph, Markart, Philipp, and Kreuter, Michael

Published
2024
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7. The role of coronary artery disease in lung transplantation: a propensity-matched analysis

Author

Lüsebrink, Enzo, Gade, Nils, Seifert, Paula, Ceelen, Felix, Veit, Tobias, Fohrer, Fabian, Hoffmann, Sabine, Höpler, Julia, Binzenhöfer, Leonhard, Roden, Daniel, Saleh, Inas, Lanz, Hugo, Michel, Sebastian, Schneider, Christian, Irlbeck, Michael, Tomasi, Roland, Hatz, Rudolf, Hausleiter, Jörg, Hagl, Christian, Magnussen, Christina, Meder, Benjamin, Zimmer, Sebastian, Luedike, Peter, Schäfer, Andreas, Orban, Martin, Milger, Katrin, Behr, Jürgen, Massberg, Steffen, and Kneidinger, Nikolaus

Published
2024
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11. High-resolution CT phenotypes in pulmonary sarcoidosis: a multinational Delphi consensus study

Author

Anderson, Adam, Morgenthau, Adam, Gaser, Adrian, Vizel, Alexander, Speranskaya, Alexandra, Gerke, Alicia, Goksel, Altinisik, Undurraga, Alvaro, Sharma, Amita, Oh, Andrea, Leung, Ann, Larici, Anna Rita, Prasse, Antje, Mazzei, Antonietta, Morais, Antonio, Bhalla, Ashu, Del Rio, Belen, Jankharia, Bhavin, Elicker, Brett, Pereira, Carlos, Biegelman-Aubry, Catherine, White, Charles, Ravaglia, Claudia, Hsia, Connie, Schaefer-Prokop, Cornelia, Launay, David, Talwar, Deepak, Castillo, Diego, Patel, Divya, Israel-Biet, Dominique, Valeyre, Dominique, Britt, Edward James, Bargagli, Elena, Bendstrup, Elisabeth, Crouser, Elliott, Nossent, Esther, Shmelev, Eugeny, Porquera, Eva Carmona, Bonella, Francesco, Cohen-Aubart, Fleur, Jeny, Florence, Ferrara, Giovanni, Jin, Gong Yong, Robbie, Hasti, Prosch, Helmut, Sumikawa, Hiromitsu, Ling-Pei, Ho, Lee, Ho Yun, Strambu, Irina, Buendia-Roldan, Ivette, Grutters, Jan, Ryu, Jay, Swigris, Jeff, Miedema, Jelle, Goo, Jin Mo, Barnett, Joseph, Verschakelen, Johny, Goldin, Jonathan, Seo, Joon Beom, Enghelmayer, Juan, Mana, Juan, Behr, Juergen, Patterson, Karen, Antoniou, Katerina, Brown, Kevin, Fujimoto, Kiminori, Savale, Laurent, Maier, Lisa, Richeldi, Luca, Neto, Manuel Lessa Ribeiro, Humbert, Marc, Judson, Marc, Veltkamp, Marcel, Wilsher, Margaret, Molina, Maria, Otaola, Maria, Revel, Marie-Pierre, Silva, Mario, Drent, Marjolein, Schiebler, Mark, Wijsenbeek-Lourens, Marlies, Bonifazi, Martina, Remy-Jardin, Martine, Koslow, Matthew, Balter, Meyer, Kreuter, Michael, Thillai, Muhunthan, Hamzeh, Nabeel, Chaudhuri, Nazia, Mogulkoc, Nesrin, Todd, Nevins, Screaton, Nick, Goh, Nicole, Shah, Nirav, Obi, Ogugua, Shlobin, Oksana, Baranova, Olga, Bresser, Paul, Rottoli, Paula, Brillet, Pierre-Yves, Said-Hartley, Qonita, Borie, Raphael, Mostard, Remy, van Zyl-smit, Richard, Kairalla, Ronaldo, Egashira, Ryoko, Boussouar, Samia, Lee, Sang Min, Bhalla, Sanjeev, Tomassetti, Sara, Quadrelli, Silvia, Hart, Simon P, Danoff, Sonye, Rajan, Sujeet, Choi, Sun Mi, Copley, Susan, Kim, Tae Jung, Johkoh, Takeshi, Corte, Tamera, McLoud, Theresa, Wessendorf, Thomas, Alfaro, Tiago, Arai, Toru, Costabel, Ulrich, Vicens-Zygmunt, Vanesa, Poletti, Venerino, Cottin, Vincent, Vucinic, Violeta, Wuyts, Wim, van Es, Wouter, Jeong, Yeon Joo, Inoue, Yoshikazu, Desai, Sujal R, Sivarasan, Nishanth, Johannson, Kerri A, George, Peter M, Culver, Daniel A, Devaraj, Anand, Lynch, David A, Milne, David, Renzoni, Elisabetta, Nunes, Hilario, Sverzellati, Nicola, Spagnolo, Paolo, Baughman, Robert P, Yadav, Ruchi, Piciucchi, Sara, Walsh, Simon L F, Kouranos, Vasileios, and Wells, Athol U

Published
2024
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13. FK506-Binding Protein 11 Is a Novel Plasma Cell-Specific Antibody Folding Catalyst with Increased Expression in Idiopathic Pulmonary Fibrosis

Author

Preisendörfer, Stefan, Ishikawa, Yoshihiro, Hennen, Elisabeth, Winklmeier, Stephan, Schupp, Jonas C, Knüppel, Larissa, Fernandez, Isis E, Binzenhöfer, Leonhard, Flatley, Andrew, Juan-Guardela, Brenda M, Ruppert, Clemens, Guenther, Andreas, Frankenberger, Marion, Hatz, Rudolf A, Kneidinger, Nikolaus, Behr, Jürgen, Feederle, Regina, Schepers, Aloys, Hilgendorff, Anne, Kaminski, Naftali, Meinl, Edgar, Bächinger, Hans Peter, Eickelberg, Oliver, and Staab-Weijnitz, Claudia A

Subjects
Biochemistry and Cell Biology, Biomedical and Clinical Sciences, Biological Sciences, Autoimmune Disease, Rare Diseases, Immunization, Lung, Respiratory, Generic health relevance, Humans, Idiopathic Pulmonary Fibrosis, Immunoglobulin G, Peptidylprolyl Isomerase, Plasma Cells, Tacrolimus Binding Proteins, antibody folding, immunophilin, lung fibrosis, interstitial lung disease, ER stress, tacrolimus, FK506-binding protein, peptidyl-prolyl isomerase, Biological sciences, Biomedical and clinical sciences
Abstract

Antibodies are central effectors of the adaptive immune response, widespread used therapeutics, but also potentially disease-causing biomolecules. Antibody folding catalysts in the plasma cell are incompletely defined. Idiopathic pulmonary fibrosis (IPF) is a fatal chronic lung disease with increasingly recognized autoimmune features. We found elevated expression of FK506-binding protein 11 (FKBP11) in IPF lungs where FKBP11 specifically localized to antibody-producing plasma cells. Suggesting a general role in plasma cells, plasma cell-specific FKBP11 expression was equally observed in lymphatic tissues, and in vitro B cell to plasma cell differentiation was accompanied by induction of FKBP11 expression. Recombinant human FKBP11 was able to refold IgG antibody in vitro and inhibited by FK506, strongly supporting a function as antibody peptidyl-prolyl cis-trans isomerase. Induction of ER stress in cell lines demonstrated induction of FKBP11 in the context of the unfolded protein response in an X-box-binding protein 1 (XBP1)-dependent manner. While deficiency of FKBP11 increased susceptibility to ER stress-mediated cell death in an alveolar epithelial cell line, FKBP11 knockdown in an antibody-producing hybridoma cell line neither induced cell death nor decreased expression or secretion of IgG antibody. Similarly, antibody secretion by the same hybridoma cell line was not affected by knockdown of the established antibody peptidyl-prolyl isomerase cyclophilin B. The results are consistent with FKBP11 as a novel XBP1-regulated antibody peptidyl-prolyl cis-trans isomerase and indicate significant redundancy in the ER-resident folding machinery of antibody-producing hybridoma cells.

Published
2022

18. Management of Acute Exacerbation of Idiopathic Pulmonary Fibrosis in Specialised and Non-specialised ILD Centres Around the World.

Author

Polke, Markus, Kondoh, Yasuhiro, Wijsenbeek, Marlies, Cottin, Vincent, Walsh, Simon, Collard, Harold, Chaudhuri, Nazia, Avdeev, Sergey, Behr, Jürgen, Calligaro, Gregory, Corte, Tamera, Flaherty, Kevin, Funke-Chambour, Manuela, Kolb, Martin, Krisam, Johannes, Maher, Toby, Molina Molina, Maria, Morais, Antonio, Moor, Catharina, Morisset, Julie, Pereira, Carlos, Quadrelli, Silvia, Selman, Moises, Tzouvelekis, Argyrios, Valenzuela, Claudia, Vancheri, Carlo, Vicens-Zygmunt, Vanesa, Wälscher, Julia, Wuyts, Wim, Bendstrup, Elisabeth, and Kreuter, Michael

Subjects
acute exacerbation, idiopathic pulmonary fibrosis, non-specialised ILD centres, pulmonologists, questionnaire, specialised ILD centres
Abstract

Background: Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is a severe complication associated with a high mortality. However, evidence and guidance on management is sparse. The aim of this international survey was to assess differences in prevention, diagnostic and treatment strategies for AE-IPF in specialised and non-specialised ILD centres worldwide. Material and Methods: Pulmonologists working in specialised and non-specialised ILD centres were invited to participate in a survey designed by an international expert panel. Responses were evaluated in respect to the physicians institutions. Results: Three hundred and two (65%) of the respondents worked in a specialised ILD centre, 134 (29%) in a non-specialised pulmonology centre. Similarities were frequent with regards to diagnostic methods including radiology and screening for infection, treatment with corticosteroids, use of high-flow oxygen and non-invasive ventilation in critical ill patients and palliative strategies. However, differences were significant in terms of the use of KL-6 and pathogen testing in urine, treatments with cyclosporine and recombinant thrombomodulin, extracorporeal membrane oxygenation in critical ill patients as well as antacid medication and anaesthesia measures as preventive methods. Conclusion: Despite the absence of recommendations, approaches to the prevention, diagnosis and treatment of AE-IPF are comparable in specialised and non-specialised ILD centres, yet certain differences in the managements of AE-IPF exist. Clinical trials and guidelines are needed to improve patient care and prognosis in AE-IPF.

Published
2021

20. Alveolar regeneration through a Krt8+ transitional stem cell state that persists in human lung fibrosis.

Author

Strunz, Maximilian, Simon, Lukas M, Ansari, Meshal, Kathiriya, Jaymin J, Angelidis, Ilias, Mayr, Christoph H, Tsidiridis, George, Lange, Marius, Mattner, Laura F, Yee, Min, Ogar, Paulina, Sengupta, Arunima, Kukhtevich, Igor, Schneider, Robert, Zhao, Zhongming, Voss, Carola, Stoeger, Tobias, Neumann, Jens HL, Hilgendorff, Anne, Behr, Jürgen, O'Reilly, Michael, Lehmann, Mareike, Burgstaller, Gerald, Königshoff, Melanie, Chapman, Harold A, Theis, Fabian J, and Schiller, Herbert B

Subjects
Pulmonary Alveoli, Stem Cells, Animals, Mice, Inbred C57BL, Humans, Mice, Pulmonary Fibrosis, Disease Models, Animal, Gene Expression Profiling, Regeneration, Cell Communication, Female, Keratin-8, Lung Injury, Single-Cell Analysis, Alveolar Epithelial Cells, Inbred C57BL, Disease Models, Animal
Abstract

The cell type specific sequences of transcriptional programs during lung regeneration have remained elusive. Using time-series single cell RNA-seq of the bleomycin lung injury model, we resolved transcriptional dynamics for 28 cell types. Trajectory modeling together with lineage tracing revealed that airway and alveolar stem cells converge on a unique Krt8 + transitional stem cell state during alveolar regeneration. These cells have squamous morphology, feature p53 and NFkB activation and display transcriptional features of cellular senescence. The Krt8+ state appears in several independent models of lung injury and persists in human lung fibrosis, creating a distinct cell-cell communication network with mesenchyme and macrophages during repair. We generated a model of gene regulatory programs leading to Krt8+ transitional cells and their terminal differentiation to alveolar type-1 cells. We propose that in lung fibrosis, perturbed molecular checkpoints on the way to terminal differentiation can cause aberrant persistence of regenerative intermediate stem cell states.

Published
2020

21. A Systematically Derived Exposure Assessment Instrument for Chronic Hypersensitivity Pneumonitis

Author

Barnes, Hayley, Morisset, Julie, Molyneaux, Philip, Westall, Glen, Glaspole, Ian, Collard, Harold R, Collaborators, CHP Exposure Assessment, Antoniou, Katerina M, Barber, Christopher M, Behr, Jürgen, Bonella, Francesco, Corte, Tamera, Costabel, Ulrich, Cottin, Vincent, Crestani, Bruno, Dalphin, Jean-Charles, Flaherty, Kevin R, Goh, Nicole, Johannson, Kerri A, Kondoh, Yasuhiro, Lederer, David, Lee, Joyce, Maher, Toby M, Martinez, Fernando J, Morell, Ferran, Noth, Imre, Raghu, Ganesh, Renzoni, Elisabetta, Richeldi, Luca, Ryerson, Christopher J, Ryu, Jay H, Salisbury, Margaret L, Singh, Sheetu, Selman, Moises, Strek, Mary E, Tarlo, Susan M, Tomassetti, Sara, Vancheri, Carlo, Vasakova, Martina, Wolters, Paul, and Wells, Athol

Subjects
Biomedical and Clinical Sciences, Clinical Sciences, Clinical Research, Management of diseases and conditions, 7.3 Management and decision making, Alveolitis, Extrinsic Allergic, Chronic Disease, Consensus, Humans, Lung, Tomography, X-Ray Computed, extrinsic allergic alveolitis, hypersensitivity pneumonitis, interstitial lung diseases, CHP Exposure Assessment Collaborators, Respiratory System, Cardiovascular medicine and haematology, Clinical sciences
Abstract

BackgroundChronic hypersensitivity pneumonitis (CHP) is an immune-mediated interstitial lung disease (ILD) caused by inhalational exposure to environmental antigens, resulting in parenchymal fibrosis. By definition, a diagnosis of CHP assumes a history of antigen exposure, but only half of all patients eventually diagnosed with CHP will have a causative antigen identified. Individual clinician variation in eliciting a history of antigen exposure may affect the frequency and confidence of CHP diagnosis.MethodsA list of potential causative exposures were derived from a systematic review of the literature. A Delphi method was applied to an international panel of ILD experts to obtain consensus regarding technique for the elicitation of exposure to antigens relevant to a diagnosis of CHP. The consensus threshold was set at 80% agreement, and median ≤ 2, interquartile range = 0 on a 5-point Likert scale (1, strongly agree; 2, tend to agree; 3, neither agree nor disagree; 4, disagree; 5, strongly disagree).ResultsIn two rounds, 36/40 experts participated. Experts agreed on 18 exposure items to ask every patient with suspected CHP. Themes included CHP inducing exposures, features that contribute to an exposure's relevance, and quantification of a relevant exposure. Based on the results from the literature review and Delphi process, a CHP exposure assessment instrument was derived. Using cognitive interviews, the instrument was revised by patients with ILD for readability and usability.ConclusionsThis Delphi survey provides items that ILD experts agree are important to ask in all patients presenting with suspected CHP and provides basis for a systematically derived CHP exposure assessment instrument. Clinical utility of this exposure assessment instrument may be affected by different local prevalence patterns of exposures. Ongoing research is required to clinically validate these items and consider their impact in more geographically diverse settings.

Published
2020

23. Proteomics reveals antiviral host response and NETosis during acute COVID-19 in high-risk patients

Author

Bauer, Alina, Pachl, Elisabeth, Hellmuth, Johannes C., Kneidinger, Nikolaus, Heydarian, Motaharehsadat, Frankenberger, Marion, Stubbe, Hans C., Ryffel, Bernhard, Petrera, Agnese, Hauck, Stefanie M., Behr, Jürgen, Kaiser, Rainer, Scherer, Clemens, Deng, Li, Teupser, Daniel, Ahmidi, Narges, Muenchhoff, Maximilian, Schubert, Benjamin, and Hilgendorff, Anne

Published
2023
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26. Risk stratification and response to therapy in patients with pulmonary arterial hypertension and comorbidities: A COMPERA analysis

Author

Rosenkranz, Stephan, Pausch, Christine, Coghlan, John G., Huscher, Doerte, Pittrow, David, Grünig, Ekkehard, Staehler, Gerd, Vizza, Carmine Dario, Gall, Henning, Distler, Oliver, Delcroix, Marion, Ghofrani, Hossain A., Ewert, Ralf, Kabitz, Hans-Joachim, Skowasch, Dirk, Behr, Juergen, Milger, Katrin, Halank, Michael, Wilkens, Heinrike, Seyfarth, Hans-Jürgen, Held, Matthias, Scelsi, Laura, Neurohr, Claus, Vonk-Noordegraaf, Anton, Ulrich, Silvia, Klose, Hans, Claussen, Martin, Eisenmann, Stephan, Schmidt, Kai-Helge, Remppis, Bjoern Andrew, Skride, Andris, Jureviciene, Elena, Gumbiene, Lina, Miliauskas, Skaidrius, Löffler-Ragg, Judith, Lange, Tobias J., Olsson, Karen M., Hoeper, Marius M., and Opitz, Christian

Published
2023
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27. Benralizumab Reduces Respiratory Exacerbations and Oral Glucocorticosteroid Dose in Patients with Severe Asthma and Eosinophilic Granulomatosis with Polyangiitis

Author

Mümmler, Carlo, primary, Mertsch, Pontus, additional, Barnikel, Michaela, additional, Haubner, Frank, additional, Schönermarck, Ulf, additional, Grabmaier, Ulrich, additional, Schulze-Koops, Hendrik, additional, Behr, Jürgen, additional, Kneidinger, Nikolaus, additional, and Milger, Katrin, additional

Published
2024
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28. Dynamics of urinary and respiratory shedding of Severe acute respiratory syndrome virus 2 (SARS-CoV-2) RNA excludes urine as a relevant source of viral transmission

Author

Mumm, Jan-Niclas, Ledderose, Stephan, Ostermann, Andreas, Rudelius, Martina, Hellmuth, Johannes C., Münchhoff, Max, Munker, Dieter, Scherer, Clemens, Volz, Yannic, Ebner, Benedikt, Giessen-Jung, Clemens, Lampert, Christopher, Vilsmaier, Theresa, Schneider, Stephanie, Gapp, Madeleine, Milger-Kneidinger, Katrin, Behr, Jürgen, von Bergwelt-Baildon, Michael, Keppler, Oliver T., Stief, Christian, Magistro, Giuseppe, Staehler, Michael, and Rodler, Severin

Published
2022
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32. Resequencing Study Confirms That Host Defense and Cell Senescence Gene Variants Contribute to the Risk of Idiopathic Pulmonary Fibrosis

Author

Moore, Camille, Blumhagen, Rachel Z, Yang, Ivana V, Walts, Avram, Powers, Julie, Walker, Tarik, Bishop, Makenna, Russell, Pamela, Vestal, Brian, Cardwell, Jonathan, Markin, Cheryl R, Mathai, Susan K, Schwarz, Marvin I, Steele, Mark P, Lee, Joyce, Brown, Kevin K, Loyd, James E, Crapo, James D, Silverman, Edwin K, Cho, Michael H, James, Judith A, Guthridge, Joel M, Cogan, Joy D, Kropski, Jonathan A, Swigris, Jeffrey J, Bair, Carol, Kim, Dong Soon, Ji, Wonjun, Kim, Hocheol, Song, Jin Woo, Maier, Lisa A, Pacheco, Karin A, Hirani, Nikhil, Poon, Azin S, Li, Feng, Jenkins, R Gisli, Braybrooke, Rebecca, Saini, Gauri, Maher, Toby M, Molyneaux, Philip L, Saunders, Peter, Zhang, Yingze, Gibson, Kevin F, Kass, Daniel J, Rojas, Mauricio, Sembrat, John, Wolters, Paul J, Collard, Harold R, Sundy, John S, O’Riordan, Thomas, Strek, Mary E, Noth, Imre, Ma, Shwu-Fan, Porteous, Mary K, Kreider, Maryl E, Patel, Namrata B, Inoue, Yoshikazu, Hirose, Masaki, Arai, Toru, Akagawa, Shinobu, Eickelberg, Oliver, Fernandez, Isis Enlil, Behr, Jürgen, Mogulkoc, Nesrin, Corte, Tamera J, Glaspole, Ian, Tomassetti, Sara, Ravaglia, Claudia, Poletti, Venerino, Crestani, Bruno, Borie, Raphael, Kannengiesser, Caroline, Parfrey, Helen, Fiddler, Christine, Rassl, Doris, Molina-Molina, Maria, Machahua, Carlos, Worboys, Ana Montes, Gudmundsson, Gunnar, Isaksson, Helgi J, Lederer, David J, Podolanczuk, Anna J, Montesi, Sydney B, Bendstrup, Elisabeth, Danchel, Vivi, Selman, Moises, Pardo, Annie, Henry, Michael T, Keane, Michael P, Doran, Peter, Vašáková, Martina, Sterclova, Martina, Ryerson, Christopher J, Wilcox, Pearce G, Okamoto, Tsukasa, Furusawa, Haruhiko, Miyazaki, Yasunari, Laurent, Geoffrey, Baltic, Svetlana, and Prele, Cecilia

Subjects
Human Genome, Autoimmune Disease, Clinical Research, Lung, Genetics, Rare Diseases, 2.1 Biological and endogenous factors, Aetiology, ATP-Binding Cassette Transporters, Case-Control Studies, Cellular Senescence, DNA Helicases, Exoribonucleases, Female, GTPase-Activating Proteins, Genetic Predisposition to Disease, Genetic Variation, Genome-Wide Association Study, High-Throughput Nucleotide Sequencing, Host-Pathogen Interactions, Humans, Idiopathic Pulmonary Fibrosis, Logistic Models, Male, Mucin-5B, Promoter Regions, Genetic, Pulmonary Surfactant-Associated Protein A, Pulmonary Surfactant-Associated Protein C, RNA, Sequence Analysis, DNA, Telomerase, Telomere-Binding Proteins, targeted resequencing, idiopathic pulmonary fibrosis, genetic variants, rare variants, disease risk alleles, Medical and Health Sciences, Respiratory System
Abstract

Rationale: Several common and rare genetic variants have been associated with idiopathic pulmonary fibrosis, a progressive fibrotic condition that is localized to the lung. Objectives: To develop an integrated understanding of the rare and common variants located in multiple loci that have been reported to contribute to the risk of disease. Methods: We performed deep targeted resequencing (3.69 Mb of DNA) in cases (n = 3,624) and control subjects (n = 4,442) across genes and regions previously associated with disease. We tested for associations between disease and 1) individual common variants via logistic regression and 2) groups of rare variants via sequence kernel association tests. Measurements and Main Results: Statistically significant common variant association signals occurred in all 10 of the regions chosen based on genome-wide association studies. The strongest risk variant is the MUC5B promoter variant rs35705950, with an odds ratio of 5.45 (95% confidence interval, 4.91-6.06) for one copy of the risk allele and 18.68 (95% confidence interval, 13.34-26.17) for two copies of the risk allele (P = 9.60 × 10-295). In addition to identifying for the first time that rare variation in FAM13A is associated with disease, we confirmed the role of rare variation in the TERT and RTEL1 gene regions in the risk of IPF, and found that the FAM13A and TERT regions have independent common and rare variant signals. Conclusions: A limited number of common and rare variants contribute to the risk of idiopathic pulmonary fibrosis in each of the resequencing regions, and these genetic variants focus on biological mechanisms of host defense and cell senescence.

Published
2019

33. Phenotyping of idiopathic pulmonary arterial hypertension: a registry analysis

Author

Hoeper, Marius M, Dwivedi, Krit, Pausch, Christine, Lewis, Robert A, Olsson, Karen M, Huscher, Doerte, Pittrow, David, Grünig, Ekkehard, Staehler, Gerd, Vizza, Carmine Dario, Gall, Henning, Distler, Oliver, Opitz, Christian, Gibbs, John Simon R, Delcroix, Marion, Park, Da-Hee, Ghofrani, Hossein Ardeschir, Ewert, Ralf, Kaemmerer, Harald, Kabitz, Hans-Joachim, Skowasch, Dirk, Behr, Juergen, Milger, Katrin, Lange, Tobias J, Wilkens, Heinrike, Seyfarth, Hans-Jürgen, Held, Matthias, Dumitrescu, Daniel, Tsangaris, Iraklis, Vonk-Noordegraaf, Anton, Ulrich, Silvia, Klose, Hans, Claussen, Martin, Eisenmann, Stephan, Schmidt, Kai-Helge, Swift, Andrew J, Thompson, Alfred A Roger, Elliot, Charlie A, Rosenkranz, Stephan, Condliffe, Robin, Kiely, David G, and Halank, Michael

Published
2022
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36. Pulmonary function impairment of asymptomatic and persistently symptomatic patients 4 months after COVID-19 according to disease severity

Author

Munker, Dieter, Veit, Tobias, Barton, Jürgen, Mertsch, Pontus, Mümmler, Carlo, Osterman, Andreas, Khatamzas, Elham, Barnikel, Michaela, Hellmuth, Johannes C., Münchhoff, Maximilian, Walter, Julia, Ghiani, Alessandro, Munker, Stefan, Dinkel, Julien, Behr, Jürgen, Kneidinger, Nikolaus, and Milger, Katrin

Published
2022
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39. A randomized controlled trial of liposomal cyclosporine A for inhalation in the prevention of bronchiolitis obliterans syndrome following lung transplantation

Author

Neurohr, Claus, Kneidinger, Nikolaus, Ghiani, Alessandro, Monforte, Víctor, Knoop, Christiane, Jaksch, Peter, Parmar, Jasvir, Ussetti, Piedad, Sole, Amparo, Müller-Quernheim, Joachim, Kessler, Romain, Wirtz, Hubert, Boerner, Gerhard, Denk, Oliver, Prante Fernandes, Stefanie, and Behr, Juergen

Published
2022
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41. Polyomavirus exerts detrimental effects on renal function in patients after lung transplantation

Author

Munker, Dieter, Veit, Tobias, Schönermarck, Ulf, Arnold, Paola, Leuschner, Gabriela, Barton, Jürgen, Mümmler, Carlo, Briegel, Ignaz, Mumm, Jan-Niclas, Zoller, Michael, Kauke, Teresa, Sisic, Alma, Ghiani, Alessandro, Walter, Julia, Milger, Katrin, Mueller, Susanna, Michel, Sebastian, Munker, Stefan, Keppler, Oliver T., Fischereder, Michael, Meiser, Bruno, Behr, Jürgen, Kneidinger, Nikolaus, and Neurohr, Claus

Published
2021
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42. Meaningful Endpoints for Idiopathic Pulmonary Fibrosis (IPF) Clinical Trials: Emphasis on ‘Feels, Functions, Survives’. Report of a Collaborative Discussion in a Symposium with Direct Engagement from Representatives of Patients, Investigators, the National Institutes of Health, a Patient Advocacy Organization, and a Regulatory Agency

Author

Raghu, Ganesh, primary, Ghazipura, Marya, additional, Fleming, Thomas R., additional, Aronson, Kerri I., additional, Behr, Jürgen, additional, Brown, Kevin K., additional, Flaherty, Kevin R., additional, Kazerooni, Ella A., additional, Maher, Toby M., additional, Richeldi, Luca, additional, Lasky, Joseph A., additional, Swigris, Jeffrey J., additional, Busch, Robert, additional, Garrard, Lili, additional, Ahn, Dong-Hyun, additional, Li, Ji, additional, Puthawala, Khalid, additional, Rodal, Gabriela, additional, Seymour, Sally, additional, Weir, Nargues, additional, Danoff, Sonye K., additional, Ettinger, Neil, additional, Goldin, Jonathan, additional, Glassberg, Marilyn K., additional, Kawano-Dourado, Leticia, additional, Khalil, Nasreen, additional, Lancaster, Lisa, additional, Lynch, David A., additional, Mageto, Yolanda, additional, Noth, Imre, additional, Shore, Jessica E., additional, Wijsenbeek, Marlies, additional, Brown, Robert, additional, Grogan, Daniel, additional, Ivey, Dorothy, additional, Golinska, Patrycja, additional, Karimi-Shah, Banu, additional, and Martinez, Fernando J., additional

Published
2024
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43. Zinpentraxin Alfa for Idiopathic Pulmonary Fibrosis: The Randomized Phase III STARSCAPE Trial

Author

Richeldi, Luca, primary, Schiffman, Courtney, additional, Behr, Jürgen, additional, Inoue, Yoshikazu, additional, Corte, Tamera J, additional, Cottin, Vincent, additional, Jenkins, R. Gisli, additional, Nathan, Steven D, additional, Raghu, Ganesh, additional, Walsh, Simon L. F., additional, Jayia, Parminderjit K., additional, Kamath, Nikhil, additional, and Martinez, Fernando J., additional

Published
2024
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44. Automated quantitative thin slice volumetric low dose CT analysis predicts disease severity in COVID-19 patients

Author

Stoleriu, Mircea Gabriel, Gerckens, Michael, Obereisenbuchner, Florian, Zaimova, Iva, Hetrodt, Justin, Mavi, Sarah-Christin, Schmidt, Felicitas, Schoenlebe, Anna Auguste, Heinig-Menhard, Katharina, Koch, Ina, Jörres, Rudolf A, Spiro, Judith, Nowak, Lorenz, Hatz, Rudolf, Behr, Jürgen, Gesierich, Wolfgang, Heiß-Neumann, Marion, and Dinkel, Julien

Published
2021
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45. Pirfenidone in patients with progressive fibrotic interstitial lung diseases other than idiopathic pulmonary fibrosis (RELIEF): a double-blind, randomised, placebo-controlled, phase 2b trial

Author

Behr, Jürgen, Kreuter, Michael, Johow, Johannes, Rabe, Klaus F, Bonella, Francesco, Bonnet, Reiner, Grohe, Christian, Held, Matthias, Wilkens, Heinrike, Hammerl, Peter, Koschel, Dirk, Blaas, Stefan, Wirtz, Hubert, Ficker, Joachim H, Neumeister, Wolfgang, Schönfeld, Nicolaus, Claussen, Martin, Kneidinger, Nikolaus, Frankenberger, Marion, Hummler, Simone, Kahn, Nicolas, Tello, Silke, Freise, Julia, Welte, Tobias, Neuser, Petra, Günther, Andreas, Schade-Brittinger, Carmen, Aminossadati, Behnaz, Nasemann, Christian, Yahiaoui, Soraya, Dupuy Backofen, Christoph, Hahmann, Maik, Wittenberg, Michael, Drakopanagiotakis, Fotis, von der Beck, Daniel, Ghofrani, Stefanie, Heinemann, Sabine, Krauss, Ekaterina, Rethorn, Hella, Koch, Andrea, Leuschner, Gabriela, Matthes, Sandhya, Neurohr, Claus, Veit, Tobias, Milger-Kneidinger, Katrin, Herth, Felix, Benstz, Julia, Bahmer, Thomas, Biller, Heike, Waschki, Benjamin, Apel, Rosa-Marie, Costabel, Ulrich, Börner, Eva, Wessendorf, Thomas, Arnrich, Margret, Ilie, Lacramioara, Wald, Alexandra, Seyfarth, Hans-Jürgen, Reinhardt, Christian, Cinar, Atif, Vogler, Markus, Huhn, Siegfried Matthias, Richter, Jaqueline, Neff, Ulrich, Blum, Torsten Gerriet, Vesenbeckh, Silvan, Boch, Christian, Semper, Hannes, Wilke, Andreas, Pfeifer, Michael, Schweda, Annette, Krill, Angelika, Lensch, Christian, Joa, Franziska, Schröder, Barbara, Plaßmeier, Annabel, Baron, Stefan, Froehling, Klaus Peter, Prasse, Antje, and Schönfeld, Nicolas

Published
2021
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46. Autoren

Author

Behr, Jürgen, primary, Böing, Sebastian, additional, Crieé, Carl, additional, Diel, Roland, additional, Frank, Wolfgang, additional, Frye, Björn C., additional, Herkenrath, Simon D., additional, Kirschbaum, Andreas, additional, Köhler, Dieter, additional, Kovacs, Zsofia, additional, Loddenkemper, Robert, additional, Melichar, Volker, additional, Müller-Quernheim, Joachim, additional, Nilius, Georg, additional, Randerath, Winfried J., additional, Rietschel, Ernst, additional, Rosewich, Martin, additional, Schaaf, Bernhard, additional, Schönhofer, Bernd, additional, Schwarz, Patrick, additional, Sebastian, Martin, additional, Stratmann, Jan A., additional, Striegel, Anne, additional, Taube, Christian, additional, Unnewehr, Markus, additional, Viniol, Christian, additional, Völkl, Thomas, additional, Wagner, Ulrich, additional, Wiater, Alfred H., additional, Wintergerst, Uwe, additional, and Zimmermann, Theodor, additional

Published
2022
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49. Efficacy and safety of sildenafil added to pirfenidone in patients with advanced idiopathic pulmonary fibrosis and risk of pulmonary hypertension: a double-blind, randomised, placebo-controlled, phase 2b trial

Author

Behr, Jürgen, Nathan, Steven D, Wuyts, Wim A, Mogulkoc Bishop, Nesrin, Bouros, Demosthenes E, Antoniou, Katerina, Guiot, Julien, Kramer, Mordechai R, Kirchgaessler, Klaus-Uwe, Bengus, Monica, Gilberg, Frank, Perjesi, Andras, Harari, Sergio, and Wells, Athol U

Published
2021
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50. Multicenter Standardization of Phase-Resolved Functional Lung MRI in Patients With Suspected Chronic Thromboembolic Pulmonary Hypertension

Author

Moher Alsady, Tawfik; https://orcid.org/0000-0002-0779-0688, Voskrebenzev, Andreas, Behrendt, Lea, Olsson, Karen, Heußel, Claus Peter, Gruenig, Ekkehard, Gall, Henning, Ghofrani, Ardeschir, Roller, Fritz, Harth, Sebastian; https://orcid.org/0000-0001-5272-2716, Marshall, Helen; https://orcid.org/0000-0002-7425-1449, Hughes, Paul J C; https://orcid.org/0000-0002-7979-5840, Wild, Jim; https://orcid.org/0000-0002-7246-8660, Swift, Andrew J, Kiely, David G, Behr, Jürgen, Dinkel, Julien, Beitzke, Dietrich; https://orcid.org/0000-0003-3179-3827, Lang, Irene M, Schmidt, Kai Helge, Kreitner, Karl Friedrich, Frauenfelder, Thomas, Ulrich, Silvia, Hamer, Okka W, Vogel-Claussen, Jens, Moher Alsady, Tawfik; https://orcid.org/0000-0002-0779-0688, Voskrebenzev, Andreas, Behrendt, Lea, Olsson, Karen, Heußel, Claus Peter, Gruenig, Ekkehard, Gall, Henning, Ghofrani, Ardeschir, Roller, Fritz, Harth, Sebastian; https://orcid.org/0000-0001-5272-2716, Marshall, Helen; https://orcid.org/0000-0002-7425-1449, Hughes, Paul J C; https://orcid.org/0000-0002-7979-5840, Wild, Jim; https://orcid.org/0000-0002-7246-8660, Swift, Andrew J, Kiely, David G, Behr, Jürgen, Dinkel, Julien, Beitzke, Dietrich; https://orcid.org/0000-0003-3179-3827, Lang, Irene M, Schmidt, Kai Helge, Kreitner, Karl Friedrich, Frauenfelder, Thomas, Ulrich, Silvia, Hamer, Okka W, and Vogel-Claussen, Jens

Abstract

BACKGROUND: Detection of pulmonary perfusion defects is the recommended approach for diagnosing chronic thromboembolic pulmonary hypertension (CTEPH). This is currently achieved in a clinical setting using scintigraphy. Phase-resolved functional lung (PREFUL) magnetic resonance imaging (MRI) is an alternative technique for evaluating regional ventilation and perfusion without the use of ionizing radiation or contrast media. PURPOSE: To assess the feasibility and image quality of PREFUL-MRI in a multicenter setting in suspected CTEPH. STUDY TYPE: This is a prospective cohort sub-study. POPULATION: Forty-five patients (64 ± 16 years old) with suspected CTEPH from nine study centers. FIELD STRENGTH/SEQUENCE: 1.5 T and 3 T/2D spoiled gradient echo/bSSFP/T2 HASTE/3D MR angiography (TWIST). ASSESSMENT: Lung signal-to-noise ratio (SNR) and contrast-to-noise ratio (CNR) were compared between study centers with different MRI machines. The contrast between normally and poorly perfused lung areas was examined on PREFUL images. The perfusion defect percentage calculated using PREFUL-MRI (QDP$_{PREFUL}$ ) was compared to QDP from the established dynamic contrast-enhanced MRI technique (QDP$_{DCE}$ ). Furthermore, QDP$_{PREFUL}$ was compared between a patient subgroup with confirmed CTEPH or chronic thromboembolic disease (CTED) to other clinical subgroups. STATISTICAL TESTS: t-Test, one-way analysis of variance (ANOVA), Pearson's correlation. Significance level was 5%. RESULTS: Significant differences in lung SNR and CNR were present between study centers. However, PREFUL perfusion images showed a significant contrast between normally and poorly perfused lung areas (mean delta of normalized perfusion -4.2% SD 3.3) with no differences between study sites (ANOVA: P = 0.065). QDP$_{PREFUL}$ was significantly correlated with QDP$_{DCE}$ (r = 0.66), and was significantly higher in 18 patients with confirmed CTEPH or CTED (57.9 ± 12.2%) compared to subgroups with other causes of PH or

Published
2024

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