Full Text Journal Articles by
Author Ulrich Costabel

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The Diagnosis and Treatment of Pulmonary Fibrosis.

Michael Kreuter, Ulf Müller Ladner, Ulrich Costabel, Danny Jonigk, Claus Peter Heussel,

<h4>Background</h4>The different types of pulmonary fibrosis are a subgroup of the interstitial lung diseases (ILDs). They are associated with a chronic and often progressive course.<h4>Methods</h4>This review is based on pertinent publications retrieved by a selective search in the EMBASE and PubMed databases, with an emphasis on articles published from 2000 ... Read more >>

Dtsch Arztebl Int (Deutsches Arzteblatt international)
[2021, 118(Forthcoming):]

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Phase 2 trial to assess lebrikizumab in patients with idiopathic pulmonary fibrosis.

Toby M Maher, Ulrich Costabel, Marilyn K Glassberg, Yasuhiro Kondoh, Takashi Ogura, Mary Beth Scholand, David Kardatzke, Monet Howard, Julie Olsson, Margaret Neighbors, Paula Belloni, Jeffrey J Swigris,

This phase 2, randomised, double-blind, placebo-controlled trial evaluated the efficacy and safety of lebrikizumab, an interleukin (IL)-13 monoclonal antibody, alone or with background pirfenidone therapy, in patients with idiopathic pulmonary fibrosis (IPF).Patients with IPF aged ≥40 years with forced vital capacity (FVC) of 40%-100% predicted and diffusing capacity for carbon monoxide ... Read more >>

Eur Respir J (The European respiratory journal)
[2021, 57(2):]

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S2K Guideline for Diagnosis of Idiopathic Pulmonary Fibrosis.

Jürgen Behr, Andreas Günther, Francesco Bonella, Julien Dinkel, Ludger Fink, Thomas Geiser, Klaus Geissler, Sven Gläser, Sabin Handzhiev, Danny Jonigk, Dirk Koschel, Michael Kreuter, Gabriela Leuschner, Philipp Markart, Antje Prasse, Nicolas Schönfeld, Jonas Christian Schupp, Helmut Sitter, Joachim Müller-Quernheim, Ulrich Costabel,

Idiopathic pulmonary fibrosis (IPF) is a severe and often fatal disease. Diagnosis of IPF requires considerable expertise and experience. Since the publication of the international IPF guideline in the year 2011 and the update 2018 several studies and technical advances have occurred, which made a new assessment of the diagnostic ... Read more >>

Respiration (Respiration; international review of thoracic diseases)
[2021, :1-34]

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Potential clinical utility of MUC5B und TOLLIP single nucleotide polymorphisms (SNPs) in the management of patients with IPF

Bonella, Ilaria Campo, Michele Zorzetto, Eda Börner, Shinichiro Ohshimo, Dirk Theegarten, Christian Taube, Ulrich Costabel,

<h4>Background: </h4> Genetic variants of TOLLIP and MUC5B, both on chromosome 11, have been reported to be associated with the development and/or prognosis of idiopathic pulmonary fibrosis (IPF). This retrospective study was conducted to investigate the association of MUC5B and TOLLIP SNPs with disease outcome in IPF. 62 IPF patients ... Read more >>

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Suggestions for improving clinical utility of future guidelines for diagnosis and management of IPF - results of a Delphi survey.

Manuela Funke-Chambour, Carlo Albera, Elisabeth Bendstrup, Ulrich Costabel, Jan C Grutters, Sergio Harari, Kerri A Johannson, Michael Kreuter, Irina Strambu, Carlo Vancheri, Francesco Varone, Patrizio Vitulo, Wim A Wuyts, Fernando Martinez, Ganesh Raghu, ,

Eur Respir J (The European respiratory journal)
[2021, :]

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A New Tool to Assess Quality of life in Patients with Idiopathic Pulmonary Fibrosis or Non-Specific Interstitial Pneumonia *

Detlef Kirsten, Ulrike de Vries, Ulrich Costabel, Dirk Koschel, Francesco Bonella, Andreas Günther, Jürgen Behr, Martin Claussen, Stefan Schwarz, Antje Prasse, Michael Kreuter,

<h4>Background: </h4> Quality of life (QoL) is significantly impaired in patients with pulmonary fibrosis, however reliable tools to assess QoL are still missing. We thus aimed to develop a new questionnaire called QPF to measure QoL in patients with fibrotic idiopathic interstitial pneumonias (IIP) . <h4>Methods:</h4> As part of a ... Read more >>

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Transbronchial cryobiopsy increases diagnostic confidence in interstitial lung disease: a prospective multicentre trial.

Jürgen Hetzel, Athol U Wells, Ulrich Costabel, Thomas V Colby, Simon L F Walsh, Johny Verschakelen, Alberto Cavazza, Sara Tomassetti, Claudia Ravaglia, Michael Böckeler, Werner Spengler, Michael Kreuter, Ralf Eberhardt, Kaid Darwiche, Alfons Torrego, Virginia Pajares, Rainer Muche, Regina Musterle, Marius Horger, Falko Fend, Arne Warth, Claus Peter Heußel, Sara Piciucchi, Alessandra Dubini, Dirk Theegarten, Tomas Franquet, Enrique Lerma, Venerino Poletti, Maik Häntschel,

<h4>Introduction</h4>The accurate diagnosis of individual interstitial lung diseases (ILD) is often challenging, but is a critical determinant of appropriate management. If a diagnosis cannot be made after multidisciplinary team discussion (MDTD), surgical lung biopsy is the current recommended tissue sampling technique according to the most recent guidelines. Transbronchial lung cryobiopsy ... Read more >>

Eur Respir J (The European respiratory journal)
[2020, 56(6):]

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Safety and tolerability of nintedanib in patients with systemic sclerosis-associated interstitial lung disease: data from the SENSCIS trial.

James R Seibold, Toby M Maher, Kristin B Highland, Shervin Assassi, Arata Azuma, Laura Kathleen Hummers, Ulrich Costabel, Ute von Wangenheim, Veronika Kohlbrenner, Martina Gahlemann, Margarida Alves, Oliver Distler, ,

<h4>Objectives</h4>To characterise the safety and tolerability of nintedanib and the dose adjustments used to manage adverse events in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD).<h4>Methods</h4>In the SENSCIS trial, patients with SSc-ILD were randomised to receive nintedanib 150 mg two times per day or placebo. To manage adverse events, treatment could ... Read more >>

Ann Rheum Dis (Annals of the rheumatic diseases)
[2020, 79(11):1478-1484]

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Progressive fibrosing interstitial lung disease: clinical uncertainties, consensus recommendations, and research priorities.

Peter M George, Paolo Spagnolo, Michael Kreuter, Goksel Altinisik, Martina Bonifazi, Fernando J Martinez, Philip L Molyneaux, Elisabetta A Renzoni, Luca Richeldi, Sara Tomassetti, Claudia Valenzuela, Carlo Vancheri, Francesco Varone, Vincent Cottin, Ulrich Costabel, ,

Within the spectrum of fibrosing interstitial lung diseases (ILDs) is a subset of patients who have inexorable progression of pulmonary fibrosis despite treatment, which is known as the progressive fibrotic phenotype. Although the concept of progressive fibrosing ILD has been applied largely to patients with idiopathic pulmonary fibrosis (IPF), there ... Read more >>

Lancet Respir Med (The Lancet. Respiratory medicine)
[2020, 8(9):925-934]

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Hypersensitivity pneumonitis.

Ulrich Costabel, Yasunari Miyazaki, Annie Pardo, Dirk Koschel, Francesco Bonella, Paolo Spagnolo, Josune Guzman, Christopher J Ryerson, Moises Selman,

Hypersensitivity pneumonitis (HP) is a complex syndrome caused by the inhalation of a variety of antigens in susceptible and sensitized individuals. These antigens are found in the environment, mostly derived from bird proteins and fungi. The prevalence and incidence of HP vary widely depending on the intensity of exposure, the ... Read more >>

Nat Rev Dis Primers (Nature reviews. Disease primers)
[2020, 6(1):65]

Cited: 2 times

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Azathioprine for Connective Tissue Disease-Associated Interstitial Lung Disease.

Eda B Boerner, Marta Cuyas, Dirk Theegarten, Shinichiro Ohshimo, Ulrich Costabel, Francesco Bonella,

<h4>Background</h4>Immunosuppressive therapy still is the standard treatment for patients with connective tissue disease-associated interstitial lung disease (CTD-ILD).<h4>Objectives</h4>This retrospective study aimed to provide data on the tolerability and efficacy of azathioprine in progressive CTD-ILDs.<h4>Methods</h4>A total of 56 patients with CTD-ILD treated with azathioprine between 2003 and 2014 were included in the ... Read more >>

Respiration (Respiration; international review of thoracic diseases)
[2020, 99(8):628-636]

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The perpetual enigma of bronchoalveolar lavage fluid lymphocytosis in chronic hypersensitivity pneumonitis: is it of diagnostic value?

Francesco Bonella, Ulrich Costabel,

Eur Respir J (The European respiratory journal)
[2020, 56(2):]

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When the Game Changes: Guidance to Adjust Sarcoidosis Management During the Coronavirus Disease 2019 Pandemic.

Nadera J Sweiss, Peter Korsten, Huzaefah J Syed, Aamer Syed, Robert P Baughman, Arthur M F Yee, Daniel A Culver, Teresa Sosenko, Arata Azuma, Francesco Bonella, Ulrich Costabel, Wonder P Drake, Marjolein Drent, Elyse E Lower, Dominique Israel-Biet, Remy L M Mostard, Hilario Nunes, Paola Rottoli, Paolo Spagnolo, Athol U Wells, Wim A Wuyts, Marc A Judson,

Chest (Chest)
[2020, 158(3):892-895]

Cited: 3 times

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Delphi consensus recommendations for a treatment algorithm in pulmonary sarcoidosis.

Franck F Rahaghi, Robert P Baughman, Lesley Ann Saketkoo, Nadera J Sweiss, Joseph B Barney, Surinder S Birring, Ulrich Costabel, Elliott D Crouser, Marjolein Drent, Alicia K Gerke, Jan C Grutters, Nabeel Y Hamzeh, Isham Huizar, W Ennis James, Sanjay Kalra, Susanna Kullberg, Huiping Li, Elyse E Lower, Lisa A Maier, Mehdi Mirsaeidi, Joachim Müller-Quernheim, Eva M Carmona Porquera, Lobelia Samavati, Dominique Valeyre, Mary Beth Scholand,

Pulmonary sarcoidosis presents substantial management challenges, with limited evidence on effective therapies and phenotypes. In the absence of definitive evidence, expert consensus can supply clinically useful guidance in medicine. An international panel of 26 experts participated in a Delphi process to identify consensus on pharmacological management in sarcoidosis with the ... Read more >>

Eur Respir Rev (European respiratory review : an official journal of the European Respiratory Society)
[2020, 29(155):]

Cited: 3 times

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A Comparison of Existing Questionnaires for Identifying the Causes of Interstitial and Rare Lung Diseases.

Markus Polke, Detlef Kirsten, Birgit Teucher, Nicolas Kahn, Klaus Geissler, Ulrich Costabel, Felix J F Herth, Michael Kreuter,

BACKGROUND:A thorough diagnostic process is essential with regard to prognosis and treatment of the more than 200 different types of interstitial lung diseases (ILD). Key to this complex process is a comprehensive medical history. For this, a template is recommended and questionnaires are increasingly used. Yet, the optimal questionnaire has ... Read more >>

Respiration (Respiration; international review of thoracic diseases)
[2020, 99(2):119-124]

Cited: 1 time

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A Systematically Derived Exposure Assessment Instrument for Chronic Hypersensitivity Pneumonitis.

Hayley Barnes, Julie Morisset, Philip Molyneaux, Glen Westall, Ian Glaspole, Harold R Collard, ,

<h4>Background</h4>Chronic 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. ... Read more >>

Chest (Chest)
[2020, 157(6):1506-1512]

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Baseline High-Resolution CT Findings Predict Acute Exacerbation of Idiopathic Pulmonary Fibrosis: German and Japanese Cohort Study.

Chihiro Hirano, Shinichiro Ohshimo, Yasushi Horimasu, Hiroshi Iwamoto, Kazunori Fujitaka, Hironobu Hamada, Nobuoki Kohno, Daisuke Komoto, Kazuo Awai, Nobuaki Shime, Francesco Bonella, Josune Guzman, Hilmar Kühl, Ulrich Costabel, Noboru Hattori,

Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is a major cause of morbidity and death in IPF. However, sensitive predictive factors of AE-IPF have not been well-investigated. To investigate whether high-resolution computed tomographic (HRCT) abnormalities predict AE-IPF in independent ethnic cohorts, this study included 121 patients with IPF (54 German ... Read more >>

J Clin Med (Journal of clinical medicine)
[2019, 8(12):]

Cited: 2 times

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Diagnostic value of BAL lymphocytosis in patients with indeterminate for usual interstitial pneumonia imaging pattern.

Vasilios Tzilas, Argyrios Tzouvelekis, Evangelos Bouros, Theodoros Karampitsakos, Maria Ntasiou, Mattheos Katsaras, Ulrich Costabel, Athol Wells, Demosthenes Bouros,

Eur Respir J (The European respiratory journal)
[2019, 54(5):]

Cited: 2 times

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Quantitative Lipidomics in Pulmonary Alveolar Proteinosis.

Matthias Griese, Francesco Bonella, Ulrich Costabel, Jacques de Blic, Nguyen-Binh Tran, Gerhard Liebisch,

<b>Rationale:</b> Pulmonary alveolar proteinosis (PAP) is characterized by filling of the alveolar spaces by lipoprotein-rich material of ill-defined composition, and is caused by molecularly different and often rare diseases that occur from birth to old age.<b>Objectives:</b> To perform a quantitative lipidomic analysis of lipids and the surfactant proteins A, B, ... Read more >>

Am J Respir Crit Care Med (American journal of respiratory and critical care medicine)
[2019, 200(7):881-887]

Cited: 5 times

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Pamrevlumab, an anti-connective tissue growth factor therapy, for idiopathic pulmonary fibrosis (PRAISE): a phase 2, randomised, double-blind, placebo-controlled trial.

Luca Richeldi, Evans R Fernández Pérez, Ulrich Costabel, Carlo Albera, David J Lederer, Kevin R Flaherty, Neil Ettinger, Rafael Perez, Mary Beth Scholand, Jonathan Goldin, Kin-Hung Peony Yu, Thomas Neff, Seth Porter, Ming Zhong, Eduard Gorina, Elias Kouchakji, Ganesh Raghu,

<h4>Background</h4>Connective tissue growth factor (CTGF) is a secreted glycoprotein that has a central role in the process of fibrosis. This study was designed to assess the safety, tolerability, and efficacy of pamrevlumab (FG-3019), a fully recombinant human monoclonal antibody against CTGF, in idiopathic pulmonary fibrosis. The aim was to establish ... Read more >>

Lancet Respir Med (The Lancet. Respiratory medicine)
[2020, 8(1):25-33]

Cited: 14 times

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Pirfenidone in patients with idiopathic pulmonary fibrosis and more advanced lung function impairment.

Steven D Nathan, Ulrich Costabel, Carlo Albera, Jürgen Behr, Wim A Wuyts, Klaus-Uwe Kirchgaessler, John L Stauffer, Elizabeth Morgenthien, Willis Chou, Susan L Limb, Paul W Noble,

<h4>Background</h4>Patients with idiopathic pulmonary fibrosis (IPF) demonstrate a range of lung function impairment. However, the efficacy of antifibrotics compared with placebo has not been assessed in patients with more advanced disease. This post-hoc analysis investigated the efficacy and safety of pirfenidone versus placebo in patients with IPF and more advanced ... Read more >>

Respir Med (Respiratory medicine)
[2019, 153:44-51]

Cited: 13 times

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Utility of Anti-DSF70 Antibodies to Predict Connective Tissue Disease in Patients Originally Presenting with Idiopathic Interstitial Pneumonia.

Yan Lyu, Eda Boerner, Dirk Theegarten, Josune Guzman, Michael Kreuter, Ulrich Costabel, Francesco Bonella,

BACKGROUND:Anti-DFS70 antibodies, corresponding to the dense fine speckled antinuclear antibody (ANA) pattern in HEp-2 substrates, have been observed in chronic inflammatory conditions, cancer and in healthy individuals but in only a small percentage of patients with connective tissue diseases (CTD). OBJECTIVES:The study was aimed to investigate the possible role of ... Read more >>

Respiration (Respiration; international review of thoracic diseases)
[2019, 98(1):29-37]

Cited: 1 time

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Effect of pirfenidone in patients with more advanced idiopathic pulmonary fibrosis.

Ulrich Costabel, Carlo Albera, Marilyn K Glassberg, Lisa H Lancaster, Wim A Wuyts, Ute Petzinger, Frank Gilberg, Klaus-Uwe Kirchgaessler, Paul W Noble,

Data from controlled clinical studies in patients with more advanced idiopathic pulmonary fibrosis (IPF) could inform clinical practice, but they are limited, since this sub-population is usually excluded from clinical trials. These exploratory post-hoc analyses of the open-label, long-term extension study RECAP (NCT00662038) aimed to assess the efficacy and safety ... Read more >>

Respir Res (Respiratory research)
[2019, 20(1):55]

Cited: 10 times

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Idiopathic interstitial pneumonia or idiopathic interstitial pneumonitis: what's in a name?

Lykourgos Kolilekas, Ulrich Costabel, Argyris Tzouvelekis, Vassilios Tzilas, Demosthenes Bouros,

Eur Respir J (The European respiratory journal)
[2019, 53(2):]

Cited: 0 times

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Efficacy of Pirfenidone in the Context of Multiple Disease Progression Events in Patients With Idiopathic Pulmonary Fibrosis.

Steven D Nathan, Ulrich Costabel, Ian Glaspole, Marilyn K Glassberg, Lisa H Lancaster, David J Lederer, Carlos A Pereira, Benjamin Trzaskoma, Elizabeth A Morgenthien, Susan L Limb, Athol U Wells,

<h4>Background</h4>Declines in percent predicted FVC (% predicted FVC), declines in 6-min walk distance (6MWD), and respiratory hospitalizations are events associated with disease progression and mortality in idiopathic pulmonary fibrosis. The incidence of multiple events in the same patient over 12 months of pirfenidone treatment is unknown.<h4>Methods</h4>Patients who received pirfenidone 2,403 mg/d (n = 623) ... Read more >>

Chest (Chest)
[2019, 155(4):712-719]

Cited: 4 times

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