Pulmonary Hypertension in Patients with Chronic Fibrosing Idiopathic Interstitial Pneumonias
Identifieur interne : 000017 ( Pmc/Curation ); précédent : 000016; suivant : 000018Pulmonary Hypertension in Patients with Chronic Fibrosing Idiopathic Interstitial Pneumonias
Auteurs : Marius M. Hoeper [Allemagne] ; Juergen Behr [Allemagne] ; Matthias Held [Allemagne] ; Ekkehard Grunig [Allemagne] ; C. Dario Vizza [Italie] ; Anton Vonk-Noordegraaf [Pays-Bas] ; Tobias J. Lange [Allemagne] ; Martin Claussen [Allemagne] ; Christian Grohé [Allemagne] ; Hans Klose [Allemagne] ; Karen M. Olsson [Allemagne] ; Thomas Zelniker [Allemagne] ; Claus Neurohr [Allemagne] ; Oliver Distler [Suisse] ; Hubert Wirtz [Allemagne] ; Christian Opitz [Allemagne] ; Doerte Huscher [Allemagne] ; David Pittrow [Allemagne] ; J. Simon R. Gibbs [Royaume-Uni]Source :
- PLoS ONE [ 1932-6203 ] ; 2015.
Abstract
Pulmonary hypertension (PH) is a common finding in patients with chronic fibrosing idiopathic interstitial pneumonias (IIP). Little is known about the response to pulmonary vasodilator therapy in this patient population. COMPERA is an international registry that prospectively captures data from patients with various forms of PH receiving pulmonary vasodilator therapies.
We retrieved data from COMPERA to compare patient characteristics, treatment patterns, response to therapy and survival in newly diagnosed patients with idiopathic pulmonary arterial hypertension (IPAH) and PH associated with IIP (PH-IIP).
Compared to patients with IPAH (n = 798), patients with PH-IIP (n = 151) were older and predominantly males. Patients with PH-IIP were treated predominantly with phosphodiesterase-5 inhibitors (88% at entry, 87% after 1 year). From baseline to the first follow-up visit, the median improvement in 6MWD was 30 m in patients with IPAH and 24.5 m in patients with PH-IIP (p = 0.457 for the difference between both groups). Improvements in NYHA functional class were observed in 22.4% and 29.5% of these patients, respectively (p = 0.179 for the difference between both groups). Survival rates were significantly worse in PH-IIP than in IPAH (3-year survival 34.0 versus 68.6%; p<0.001). Total lung capacity, NYHA class IV, and mixed-venous oxygen saturation were independent predictors of survival in patients with PH-IIP.
Patients with PH-IIP have a dismal prognosis. Our results suggest that pulmonary vasodilator therapy may be associated with short-term functional improvement in some of these patients but it is unclear whether this treatment affects survival.
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DOI: 10.1371/journal.pone.0141911
PubMed: 26630396
PubMed Central: 4667900
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<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Pulmonary Hypertension in Patients with Chronic Fibrosing Idiopathic Interstitial Pneumonias</title><author><name sortKey="Hoeper, Marius M" sort="Hoeper, Marius M" uniqKey="Hoeper M" first="Marius M." last="Hoeper">Marius M. Hoeper</name><affiliation wicri:level="1"><nlm:aff id="aff001"><addr-line>Department of Respiratory Medicine and German Center of Lung Research (DZL), Hannover Medical School, Hannover, Germany</addr-line></nlm:aff><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Respiratory Medicine and German Center of Lung Research (DZL), Hannover Medical School, Hannover</wicri:regionArea></affiliation></author><author><name sortKey="Behr, Juergen" sort="Behr, Juergen" uniqKey="Behr J" first="Juergen" last="Behr">Juergen Behr</name><affiliation wicri:level="1"><nlm:aff id="aff002"><addr-line>Department of Internal Medicine V, University of Munich, Munich, Germany</addr-line></nlm:aff><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Internal Medicine V, University of Munich, Munich</wicri:regionArea></affiliation></author><author><name sortKey="Held, Matthias" sort="Held, Matthias" uniqKey="Held M" first="Matthias" last="Held">Matthias Held</name><affiliation wicri:level="1"><nlm:aff id="aff003"><addr-line>Department of Internal Medicine, Respiratory Medicine and Cardiology, Mission Medical Hospital, Würzburg, Germany</addr-line></nlm:aff><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Internal Medicine, Respiratory Medicine and Cardiology, Mission Medical Hospital, Würzburg</wicri:regionArea></affiliation></author><author><name sortKey="Grunig, Ekkehard" sort="Grunig, Ekkehard" uniqKey="Grunig E" first="Ekkehard" last="Grunig">Ekkehard Grunig</name><affiliation wicri:level="1"><nlm:aff id="aff004"><addr-line>University Hospital Heidelberg, Heidelberg, Germany</addr-line></nlm:aff><country xml:lang="fr">Allemagne</country><wicri:regionArea>University Hospital Heidelberg, Heidelberg</wicri:regionArea></affiliation></author><author><name sortKey="Vizza, C Dario" sort="Vizza, C Dario" uniqKey="Vizza C" first="C. 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Olsson</name><affiliation wicri:level="1"><nlm:aff id="aff001"><addr-line>Department of Respiratory Medicine and German Center of Lung Research (DZL), Hannover Medical School, Hannover, Germany</addr-line></nlm:aff><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Respiratory Medicine and German Center of Lung Research (DZL), Hannover Medical School, Hannover</wicri:regionArea></affiliation></author><author><name sortKey="Zelniker, Thomas" sort="Zelniker, Thomas" uniqKey="Zelniker T" first="Thomas" last="Zelniker">Thomas Zelniker</name><affiliation wicri:level="1"><nlm:aff id="aff011"><addr-line>Department of Cardiology, Angiology and Pneumology, University of Heidelberg, Heidelberg, Germany</addr-line></nlm:aff><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Cardiology, Angiology and Pneumology, University of Heidelberg, Heidelberg</wicri:regionArea></affiliation></author><author><name sortKey="Neurohr, Claus" sort="Neurohr, Claus" uniqKey="Neurohr C" first="Claus" last="Neurohr">Claus Neurohr</name><affiliation wicri:level="1"><nlm:aff id="aff002"><addr-line>Department of Internal Medicine V, University of Munich, Munich, Germany</addr-line></nlm:aff><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Internal Medicine V, University of Munich, Munich</wicri:regionArea></affiliation></author><author><name sortKey="Distler, Oliver" sort="Distler, Oliver" uniqKey="Distler O" first="Oliver" last="Distler">Oliver Distler</name><affiliation wicri:level="1"><nlm:aff id="aff012"><addr-line>Division of Rheumatology, University Hospital Zurich, Zurich, Switzerland</addr-line></nlm:aff><country xml:lang="fr">Suisse</country><wicri:regionArea>Division of Rheumatology, University Hospital Zurich, Zurich</wicri:regionArea></affiliation></author><author><name sortKey="Wirtz, Hubert" sort="Wirtz, Hubert" uniqKey="Wirtz H" first="Hubert" last="Wirtz">Hubert Wirtz</name><affiliation wicri:level="1"><nlm:aff id="aff013"><addr-line>Department of Respiratory Medicine, University of Leipzig, Leipzig, Germany</addr-line></nlm:aff><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Respiratory Medicine, University of Leipzig, Leipzig</wicri:regionArea></affiliation></author><author><name sortKey="Opitz, Christian" sort="Opitz, Christian" uniqKey="Opitz C" first="Christian" last="Opitz">Christian Opitz</name><affiliation wicri:level="1"><nlm:aff id="aff014"><addr-line>Department of Cardiology, DRK Kliniken Berlin Köpenick, Berlin, Germany</addr-line></nlm:aff><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Cardiology, DRK Kliniken Berlin Köpenick, Berlin</wicri:regionArea></affiliation></author><author><name sortKey="Huscher, Doerte" sort="Huscher, Doerte" uniqKey="Huscher D" first="Doerte" last="Huscher">Doerte Huscher</name><affiliation wicri:level="1"><nlm:aff id="aff015"><addr-line>Department of Rheumatology and Clinical Immunology, Charité University Hospital, and Epidemiology unit, German Rheumatism Research Centre, Berlin, Germany</addr-line></nlm:aff><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Rheumatology and Clinical Immunology, Charité University Hospital, and Epidemiology unit, German Rheumatism Research Centre, Berlin</wicri:regionArea></affiliation></author><author><name sortKey="Pittrow, David" sort="Pittrow, David" uniqKey="Pittrow D" first="David" last="Pittrow">David Pittrow</name><affiliation wicri:level="1"><nlm:aff id="aff016"><addr-line>Institute for Clinical Pharmacology, Medical Faculty, Technical University, Dresden, Germany</addr-line></nlm:aff><country xml:lang="fr">Allemagne</country><wicri:regionArea>Institute for Clinical Pharmacology, Medical Faculty, Technical University, Dresden</wicri:regionArea></affiliation></author><author><name sortKey="Gibbs, J Simon R" sort="Gibbs, J Simon R" uniqKey="Gibbs J" first="J. Simon R." last="Gibbs">J. Simon R. Gibbs</name><affiliation wicri:level="1"><nlm:aff id="aff017"><addr-line>Department of Cardiology, National Heart & Lung Institute; Imperial College London, London, United Kingdom</addr-line></nlm:aff><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Department of Cardiology, National Heart & Lung Institute; Imperial College London, London</wicri:regionArea></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">PMC</idno><idno type="pmid">26630396</idno><idno type="pmc">4667900</idno><idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4667900</idno><idno type="RBID">PMC:4667900</idno><idno type="doi">10.1371/journal.pone.0141911</idno><date when="2015">2015</date><idno type="wicri:Area/Pmc/Corpus">000017</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Corpus" wicri:corpus="PMC">000017</idno><idno type="wicri:Area/Pmc/Curation">000017</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Curation">000017</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">Pulmonary Hypertension in Patients with Chronic Fibrosing Idiopathic Interstitial Pneumonias</title><author><name sortKey="Hoeper, Marius M" sort="Hoeper, Marius M" uniqKey="Hoeper M" first="Marius M." last="Hoeper">Marius M. Hoeper</name><affiliation wicri:level="1"><nlm:aff id="aff001"><addr-line>Department of Respiratory Medicine and German Center of Lung Research (DZL), Hannover Medical School, Hannover, Germany</addr-line></nlm:aff><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Respiratory Medicine and German Center of Lung Research (DZL), Hannover Medical School, Hannover</wicri:regionArea></affiliation></author><author><name sortKey="Behr, Juergen" sort="Behr, Juergen" uniqKey="Behr J" first="Juergen" last="Behr">Juergen Behr</name><affiliation wicri:level="1"><nlm:aff id="aff002"><addr-line>Department of Internal Medicine V, University of Munich, Munich, Germany</addr-line></nlm:aff><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Internal Medicine V, University of Munich, Munich</wicri:regionArea></affiliation></author><author><name sortKey="Held, Matthias" sort="Held, Matthias" uniqKey="Held M" first="Matthias" last="Held">Matthias Held</name><affiliation wicri:level="1"><nlm:aff id="aff003"><addr-line>Department of Internal Medicine, Respiratory Medicine and Cardiology, Mission Medical Hospital, Würzburg, Germany</addr-line></nlm:aff><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Internal Medicine, Respiratory Medicine and Cardiology, Mission Medical Hospital, Würzburg</wicri:regionArea></affiliation></author><author><name sortKey="Grunig, Ekkehard" sort="Grunig, Ekkehard" uniqKey="Grunig E" first="Ekkehard" last="Grunig">Ekkehard Grunig</name><affiliation wicri:level="1"><nlm:aff id="aff004"><addr-line>University Hospital Heidelberg, Heidelberg, Germany</addr-line></nlm:aff><country xml:lang="fr">Allemagne</country><wicri:regionArea>University Hospital Heidelberg, Heidelberg</wicri:regionArea></affiliation></author><author><name sortKey="Vizza, C Dario" sort="Vizza, C Dario" uniqKey="Vizza C" first="C. Dario" last="Vizza">C. Dario Vizza</name><affiliation wicri:level="1"><nlm:aff id="aff005"><addr-line>Department of Cardiovascular and Respiratory Diseases, Sapienza, University of Rome, Rome, Italy</addr-line></nlm:aff><country xml:lang="fr">Italie</country><wicri:regionArea>Department of Cardiovascular and Respiratory Diseases, Sapienza, University of Rome, Rome</wicri:regionArea></affiliation></author><author><name sortKey="Vonk Noordegraaf, Anton" sort="Vonk Noordegraaf, Anton" uniqKey="Vonk Noordegraaf A" first="Anton" last="Vonk-Noordegraaf">Anton Vonk-Noordegraaf</name><affiliation wicri:level="1"><nlm:aff id="aff006"><addr-line>Department of Pulmonary Diseases, VU University Medical Center, Amsterdam, The Netherlands</addr-line></nlm:aff><country xml:lang="fr">Pays-Bas</country><wicri:regionArea>Department of Pulmonary Diseases, VU University Medical Center, Amsterdam</wicri:regionArea></affiliation></author><author><name sortKey="Lange, Tobias J" sort="Lange, Tobias J" uniqKey="Lange T" first="Tobias J." last="Lange">Tobias J. Lange</name><affiliation wicri:level="1"><nlm:aff id="aff007"><addr-line>Department of Internal Medicine II, Division of Pneumology, University Medical Center Regensburg, Regensburg, Germany</addr-line></nlm:aff><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Internal Medicine II, Division of Pneumology, University Medical Center Regensburg, Regensburg</wicri:regionArea></affiliation></author><author><name sortKey="Claussen, Martin" sort="Claussen, Martin" uniqKey="Claussen M" first="Martin" last="Claussen">Martin Claussen</name><affiliation wicri:level="1"><nlm:aff id="aff008"><addr-line>LungenClinic, Grosshansdorf, Germany</addr-line></nlm:aff><country xml:lang="fr">Allemagne</country><wicri:regionArea>LungenClinic, Grosshansdorf</wicri:regionArea></affiliation></author><author><name sortKey="Grohe, Christian" sort="Grohe, Christian" uniqKey="Grohe C" first="Christian" last="Grohé">Christian Grohé</name><affiliation wicri:level="1"><nlm:aff id="aff009"><addr-line>Department of Respiratory Medicine, ELK Thorax Centre, Berlin, Germany</addr-line></nlm:aff><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Respiratory Medicine, ELK Thorax Centre, Berlin</wicri:regionArea></affiliation></author><author><name sortKey="Klose, Hans" sort="Klose, Hans" uniqKey="Klose H" first="Hans" last="Klose">Hans Klose</name><affiliation wicri:level="1"><nlm:aff id="aff010"><addr-line>University Medical Center Hamburg-Eppendorf, Center of Oncology, Department of Respiratory Medicine, Hamburg, Germany</addr-line></nlm:aff><country xml:lang="fr">Allemagne</country><wicri:regionArea>University Medical Center Hamburg-Eppendorf, Center of Oncology, Department of Respiratory Medicine, Hamburg</wicri:regionArea></affiliation></author><author><name sortKey="Olsson, Karen M" sort="Olsson, Karen M" uniqKey="Olsson K" first="Karen M." last="Olsson">Karen M. Olsson</name><affiliation wicri:level="1"><nlm:aff id="aff001"><addr-line>Department of Respiratory Medicine and German Center of Lung Research (DZL), Hannover Medical School, Hannover, Germany</addr-line></nlm:aff><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Respiratory Medicine and German Center of Lung Research (DZL), Hannover Medical School, Hannover</wicri:regionArea></affiliation></author><author><name sortKey="Zelniker, Thomas" sort="Zelniker, Thomas" uniqKey="Zelniker T" first="Thomas" last="Zelniker">Thomas Zelniker</name><affiliation wicri:level="1"><nlm:aff id="aff011"><addr-line>Department of Cardiology, Angiology and Pneumology, University of Heidelberg, Heidelberg, Germany</addr-line></nlm:aff><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Cardiology, Angiology and Pneumology, University of Heidelberg, Heidelberg</wicri:regionArea></affiliation></author><author><name sortKey="Neurohr, Claus" sort="Neurohr, Claus" uniqKey="Neurohr C" first="Claus" last="Neurohr">Claus Neurohr</name><affiliation wicri:level="1"><nlm:aff id="aff002"><addr-line>Department of Internal Medicine V, University of Munich, Munich, Germany</addr-line></nlm:aff><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Internal Medicine V, University of Munich, Munich</wicri:regionArea></affiliation></author><author><name sortKey="Distler, Oliver" sort="Distler, Oliver" uniqKey="Distler O" first="Oliver" last="Distler">Oliver Distler</name><affiliation wicri:level="1"><nlm:aff id="aff012"><addr-line>Division of Rheumatology, University Hospital Zurich, Zurich, Switzerland</addr-line></nlm:aff><country xml:lang="fr">Suisse</country><wicri:regionArea>Division of Rheumatology, University Hospital Zurich, Zurich</wicri:regionArea></affiliation></author><author><name sortKey="Wirtz, Hubert" sort="Wirtz, Hubert" uniqKey="Wirtz H" first="Hubert" last="Wirtz">Hubert Wirtz</name><affiliation wicri:level="1"><nlm:aff id="aff013"><addr-line>Department of Respiratory Medicine, University of Leipzig, Leipzig, Germany</addr-line></nlm:aff><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Respiratory Medicine, University of Leipzig, Leipzig</wicri:regionArea></affiliation></author><author><name sortKey="Opitz, Christian" sort="Opitz, Christian" uniqKey="Opitz C" first="Christian" last="Opitz">Christian Opitz</name><affiliation wicri:level="1"><nlm:aff id="aff014"><addr-line>Department of Cardiology, DRK Kliniken Berlin Köpenick, Berlin, Germany</addr-line></nlm:aff><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Cardiology, DRK Kliniken Berlin Köpenick, Berlin</wicri:regionArea></affiliation></author><author><name sortKey="Huscher, Doerte" sort="Huscher, Doerte" uniqKey="Huscher D" first="Doerte" last="Huscher">Doerte Huscher</name><affiliation wicri:level="1"><nlm:aff id="aff015"><addr-line>Department of Rheumatology and Clinical Immunology, Charité University Hospital, and Epidemiology unit, German Rheumatism Research Centre, Berlin, Germany</addr-line></nlm:aff><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Rheumatology and Clinical Immunology, Charité University Hospital, and Epidemiology unit, German Rheumatism Research Centre, Berlin</wicri:regionArea></affiliation></author><author><name sortKey="Pittrow, David" sort="Pittrow, David" uniqKey="Pittrow D" first="David" last="Pittrow">David Pittrow</name><affiliation wicri:level="1"><nlm:aff id="aff016"><addr-line>Institute for Clinical Pharmacology, Medical Faculty, Technical University, Dresden, Germany</addr-line></nlm:aff><country xml:lang="fr">Allemagne</country><wicri:regionArea>Institute for Clinical Pharmacology, Medical Faculty, Technical University, Dresden</wicri:regionArea></affiliation></author><author><name sortKey="Gibbs, J Simon R" sort="Gibbs, J Simon R" uniqKey="Gibbs J" first="J. Simon R." last="Gibbs">J. Simon R. Gibbs</name><affiliation wicri:level="1"><nlm:aff id="aff017"><addr-line>Department of Cardiology, National Heart & Lung Institute; Imperial College London, London, United Kingdom</addr-line></nlm:aff><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Department of Cardiology, National Heart & Lung Institute; Imperial College London, London</wicri:regionArea></affiliation></author></analytic><series><title level="j">PLoS ONE</title><idno type="eISSN">1932-6203</idno><imprint><date when="2015">2015</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en"><sec id="sec001"><title>Background</title><p>Pulmonary hypertension (PH) is a common finding in patients with chronic fibrosing idiopathic interstitial pneumonias (IIP). Little is known about the response to pulmonary vasodilator therapy in this patient population. COMPERA is an international registry that prospectively captures data from patients with various forms of PH receiving pulmonary vasodilator therapies.</p></sec><sec id="sec002"><title>Methods</title><p>We retrieved data from COMPERA to compare patient characteristics, treatment patterns, response to therapy and survival in newly diagnosed patients with idiopathic pulmonary arterial hypertension (IPAH) and PH associated with IIP (PH-IIP).</p></sec><sec id="sec003"><title>Results</title><p>Compared to patients with IPAH (n = 798), patients with PH-IIP (n = 151) were older and predominantly males. Patients with PH-IIP were treated predominantly with phosphodiesterase-5 inhibitors (88% at entry, 87% after 1 year). From baseline to the first follow-up visit, the median improvement in 6MWD was 30 m in patients with IPAH and 24.5 m in patients with PH-IIP (p = 0.457 for the difference between both groups). Improvements in NYHA functional class were observed in 22.4% and 29.5% of these patients, respectively (p = 0.179 for the difference between both groups). Survival rates were significantly worse in PH-IIP than in IPAH (3-year survival 34.0 versus 68.6%; p<0.001). Total lung capacity, NYHA class IV, and mixed-venous oxygen saturation were independent predictors of survival in patients with PH-IIP.</p></sec><sec id="sec004"><title>Conclusions</title><p>Patients with PH-IIP have a dismal prognosis. Our results suggest that pulmonary vasodilator therapy may be associated with short-term functional improvement in some of these patients but it is unclear whether this treatment affects survival.</p></sec><sec id="sec005"><title>Trial Registration</title><p>clinicaltrials.gov <ext-link ext-link-type="uri" xlink:href="https://clinicaltrials.gov/ct2/show/NCT01347216">NCT01347216</ext-link></p></sec></div></front><back><div1 type="bibliography"><listBibl><biblStruct><analytic><author><name sortKey="Travis, Wd" uniqKey="Travis W">WD Travis</name></author><author><name sortKey="Costabel, U" uniqKey="Costabel U">U Costabel</name></author><author><name sortKey="Hansell, Dm" uniqKey="Hansell D">DM Hansell</name></author><author><name sortKey="King, Te" uniqKey="King T">TE King</name></author><author><name sortKey="Lynch, Da" uniqKey="Lynch D">DA Lynch</name></author><author><name sortKey="Nicholson, Ag" uniqKey="Nicholson A">AG Nicholson</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Behr, J" uniqKey="Behr J">J Behr</name></author><author><name sortKey="Ryu, Jh" uniqKey="Ryu J">JH Ryu</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Seeger, W" uniqKey="Seeger W">W Seeger</name></author><author><name sortKey="Adir, Y" uniqKey="Adir Y">Y Adir</name></author><author><name sortKey="Barbera, Ja" uniqKey="Barbera J">JA Barbera</name></author><author><name sortKey="Champion, H" uniqKey="Champion H">H Champion</name></author><author><name sortKey="Coghlan, Jg" uniqKey="Coghlan J">JG Coghlan</name></author><author><name sortKey="Cottin, V" uniqKey="Cottin V">V Cottin</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Behr, J" uniqKey="Behr J">J Behr</name></author><author><name sortKey="Kreuter, M" uniqKey="Kreuter M">M Kreuter</name></author><author><name sortKey="Hoeper, Mm" uniqKey="Hoeper M">MM Hoeper</name></author><author><name sortKey="Wirtz, H" uniqKey="Wirtz H">H Wirtz</name></author><author><name sortKey="Klotsche, J" uniqKey="Klotsche J">J Klotsche</name></author><author><name sortKey="Koschel, D" uniqKey="Koschel D">D Koschel</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Hamada, K" uniqKey="Hamada K">K Hamada</name></author><author><name sortKey="Nagai, S" uniqKey="Nagai S">S Nagai</name></author><author><name sortKey="Tanaka, S" uniqKey="Tanaka S">S Tanaka</name></author><author><name sortKey="Handa, T" uniqKey="Handa T">T Handa</name></author><author><name sortKey="Shigematsu, M" uniqKey="Shigematsu M">M Shigematsu</name></author><author><name sortKey="Nagao, T" uniqKey="Nagao T">T Nagao</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Nathan, Sd" uniqKey="Nathan S">SD Nathan</name></author><author><name sortKey="Shlobin, Oa" uniqKey="Shlobin O">OA Shlobin</name></author><author><name sortKey="Ahmad, S" uniqKey="Ahmad S">S Ahmad</name></author><author><name sortKey="Koch, J" uniqKey="Koch J">J Koch</name></author><author><name sortKey="Barnett, Sd" uniqKey="Barnett S">SD Barnett</name></author><author><name sortKey="Ad, N" uniqKey="Ad N">N Ad</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Shorr, Af" uniqKey="Shorr A">AF Shorr</name></author><author><name sortKey="Wainright, Jl" uniqKey="Wainright J">JL Wainright</name></author><author><name sortKey="Cors, Cs" uniqKey="Cors C">CS Cors</name></author><author><name sortKey="Lettieri, Cj" uniqKey="Lettieri C">CJ Lettieri</name></author><author><name sortKey="Nathan, Sd" uniqKey="Nathan S">SD Nathan</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Minai, Oa" uniqKey="Minai O">OA Minai</name></author><author><name sortKey="Santacruz, Jf" uniqKey="Santacruz J">JF Santacruz</name></author><author><name sortKey="Alster, Jm" uniqKey="Alster J">JM Alster</name></author><author><name sortKey="Budev, Mm" uniqKey="Budev M">MM Budev</name></author><author><name sortKey="Mccarthy, K" uniqKey="Mccarthy K">K McCarthy</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Kimura, M" uniqKey="Kimura M">M Kimura</name></author><author><name sortKey="Taniguchi, H" uniqKey="Taniguchi H">H Taniguchi</name></author><author><name sortKey="Kondoh, Y" uniqKey="Kondoh Y">Y Kondoh</name></author><author><name sortKey="Kimura, T" uniqKey="Kimura T">T Kimura</name></author><author><name sortKey="Kataoka, K" uniqKey="Kataoka K">K Kataoka</name></author><author><name sortKey="Nishiyama, O" uniqKey="Nishiyama O">O Nishiyama</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Lettieri, Cj" uniqKey="Lettieri C">CJ Lettieri</name></author><author><name sortKey="Nathan, Sd" uniqKey="Nathan S">SD Nathan</name></author><author><name sortKey="Barnett, Sd" uniqKey="Barnett S">SD Barnett</name></author><author><name sortKey="Ahmad, S" uniqKey="Ahmad S">S Ahmad</name></author><author><name sortKey="Shorr, Af" uniqKey="Shorr A">AF Shorr</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Mejia, M" uniqKey="Mejia M">M Mejia</name></author><author><name sortKey="Carrillo, G" uniqKey="Carrillo G">G Carrillo</name></author><author><name sortKey="Rojas Serrano, J" uniqKey="Rojas Serrano J">J Rojas-Serrano</name></author><author><name sortKey="Estrada, A" uniqKey="Estrada A">A Estrada</name></author><author><name sortKey="Suarez, T" uniqKey="Suarez T">T Suarez</name></author><author><name sortKey="Alonso, D" uniqKey="Alonso D">D Alonso</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Corte, Tj" uniqKey="Corte T">TJ Corte</name></author><author><name sortKey="Wort, Sj" uniqKey="Wort S">SJ Wort</name></author><author><name sortKey="Gatzoulis, Ma" uniqKey="Gatzoulis M">MA Gatzoulis</name></author><author><name sortKey="Macdonald, P" uniqKey="Macdonald P">P Macdonald</name></author><author><name sortKey="Hansell, Dm" uniqKey="Hansell D">DM Hansell</name></author><author><name sortKey="Wells, Au" uniqKey="Wells A">AU Wells</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Rivera Lebron, Bn" uniqKey="Rivera Lebron B">BN Rivera-Lebron</name></author><author><name sortKey="Forfia, Pr" uniqKey="Forfia P">PR Forfia</name></author><author><name sortKey="Kreider, M" uniqKey="Kreider M">M Kreider</name></author><author><name sortKey="Lee, Jc" uniqKey="Lee J">JC Lee</name></author><author><name sortKey="Holmes, Jh" uniqKey="Holmes J">JH Holmes</name></author><author><name sortKey="Kawut, Sm" uniqKey="Kawut S">SM Kawut</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Humbert, M" uniqKey="Humbert M">M Humbert</name></author><author><name sortKey="Lau, Em" uniqKey="Lau E">EM Lau</name></author><author><name sortKey="Montani, D" uniqKey="Montani D">D Montani</name></author><author><name sortKey="Jais, X" uniqKey="Jais X">X Jais</name></author><author><name sortKey="Sitbon, O" uniqKey="Sitbon O">O Sitbon</name></author><author><name sortKey="Simonneau, G" uniqKey="Simonneau G">G Simonneau</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Corte, Tj" uniqKey="Corte T">TJ Corte</name></author><author><name sortKey="Keir, Gj" uniqKey="Keir G">GJ Keir</name></author><author><name sortKey="Dimopoulos, K" uniqKey="Dimopoulos K">K Dimopoulos</name></author><author><name sortKey="Howard, L" uniqKey="Howard L">L Howard</name></author><author><name sortKey="Corris, Pa" uniqKey="Corris P">PA Corris</name></author><author><name sortKey="Parfitt, L" uniqKey="Parfitt L">L Parfitt</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Zisman, Da" uniqKey="Zisman D">DA Zisman</name></author><author><name sortKey="Schwarz, M" uniqKey="Schwarz M">M Schwarz</name></author><author><name sortKey="Anstrom, Kj" uniqKey="Anstrom K">KJ Anstrom</name></author><author><name sortKey="Collard, Hr" uniqKey="Collard H">HR Collard</name></author><author><name sortKey="Flaherty, Kr" uniqKey="Flaherty K">KR Flaherty</name></author><author><name sortKey="Hunninghake, Gw" uniqKey="Hunninghake G">GW Hunninghake</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Hoeper, Mm" uniqKey="Hoeper M">MM Hoeper</name></author><author><name sortKey="Andreas, S" uniqKey="Andreas S">S Andreas</name></author><author><name sortKey="Bastian, A" uniqKey="Bastian A">A Bastian</name></author><author><name sortKey="Claussen, M" uniqKey="Claussen M">M Claussen</name></author><author><name sortKey="Ghofrani, Ha" uniqKey="Ghofrani H">HA Ghofrani</name></author><author><name sortKey="Gorenflo, M" uniqKey="Gorenflo M">M Gorenflo</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Galie, N" uniqKey="Galie N">N Galie</name></author><author><name sortKey="Hoeper, Mm" uniqKey="Hoeper M">MM Hoeper</name></author><author><name sortKey="Humbert, M" uniqKey="Humbert M">M Humbert</name></author><author><name sortKey="Torbicki, A" uniqKey="Torbicki A">A Torbicki</name></author><author><name sortKey="Vachiery, Jl" uniqKey="Vachiery J">JL Vachiery</name></author><author><name sortKey="Barbera, Ja" uniqKey="Barbera J">JA Barbera</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Tanabe, N" uniqKey="Tanabe N">N Tanabe</name></author><author><name sortKey="Taniguchi, H" uniqKey="Taniguchi H">H Taniguchi</name></author><author><name sortKey="Tsujino, I" uniqKey="Tsujino I">I Tsujino</name></author><author><name sortKey="Sakamaki, F" uniqKey="Sakamaki F">F Sakamaki</name></author><author><name sortKey="Emoto, N" uniqKey="Emoto N">N Emoto</name></author><author><name sortKey="Kimura, H" uniqKey="Kimura H">H Kimura</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Hurdman, J" uniqKey="Hurdman J">J Hurdman</name></author><author><name sortKey="Condliffe, R" uniqKey="Condliffe R">R Condliffe</name></author><author><name sortKey="Elliot, Ca" uniqKey="Elliot C">CA Elliot</name></author><author><name sortKey="Swift, A" uniqKey="Swift A">A Swift</name></author><author><name sortKey="Rajaram, S" uniqKey="Rajaram S">S Rajaram</name></author><author><name sortKey="Davies, C" uniqKey="Davies C">C Davies</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Hurdman, J" uniqKey="Hurdman J">J Hurdman</name></author><author><name sortKey="Condliffe, R" uniqKey="Condliffe R">R Condliffe</name></author><author><name sortKey="Elliot, Ca" uniqKey="Elliot C">CA Elliot</name></author><author><name sortKey="Davies, C" uniqKey="Davies C">C Davies</name></author><author><name sortKey="Hill, C" uniqKey="Hill C">C Hill</name></author><author><name sortKey="Wild, Jm" uniqKey="Wild J">JM Wild</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Brewis, Mj" uniqKey="Brewis M">MJ Brewis</name></author><author><name sortKey="Church, Ac" uniqKey="Church A">AC Church</name></author><author><name sortKey="Johnson, Mk" uniqKey="Johnson M">MK Johnson</name></author><author><name sortKey="Peacock, Aj" uniqKey="Peacock A">AJ Peacock</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="King, Te" uniqKey="King T">TE King</name></author><author><name sortKey="Behr, J" uniqKey="Behr J">J Behr</name></author><author><name sortKey="Brown, Kk" uniqKey="Brown K">KK Brown</name></author><author><name sortKey="Du Bois, Rm" uniqKey="Du Bois R">RM du Bois</name></author><author><name sortKey="Lancaster, L" uniqKey="Lancaster L">L Lancaster</name></author><author><name sortKey="De Andrade, Ja" uniqKey="De Andrade J">JA de Andrade</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="King, Te" uniqKey="King T">TE King</name></author><author><name sortKey="Brown, Kk" uniqKey="Brown K">KK Brown</name></author><author><name sortKey="Raghu, G" uniqKey="Raghu G">G Raghu</name></author><author><name sortKey="Du Bois, Rm" uniqKey="Du Bois R">RM du Bois</name></author><author><name sortKey="Lynch, Da" uniqKey="Lynch D">DA Lynch</name></author><author><name sortKey="Martinez, F" uniqKey="Martinez F">F Martinez</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Seibold, Jr" uniqKey="Seibold J">JR Seibold</name></author><author><name sortKey="Denton, Cp" uniqKey="Denton C">CP Denton</name></author><author><name sortKey="Furst, De" uniqKey="Furst D">DE Furst</name></author><author><name sortKey="Guillevin, L" uniqKey="Guillevin L">L Guillevin</name></author><author><name sortKey="Rubin, Lj" uniqKey="Rubin L">LJ Rubin</name></author><author><name sortKey="Wells, A" uniqKey="Wells A">A Wells</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Raghu, G" uniqKey="Raghu G">G Raghu</name></author><author><name sortKey="Behr, J" uniqKey="Behr J">J Behr</name></author><author><name sortKey="Brown, Kk" uniqKey="Brown K">KK Brown</name></author><author><name sortKey="Egan, Jj" uniqKey="Egan J">JJ Egan</name></author><author><name sortKey="Kawut, Sm" uniqKey="Kawut S">SM Kawut</name></author><author><name sortKey="Flaherty, Kr" uniqKey="Flaherty K">KR Flaherty</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Raghu, G" uniqKey="Raghu G">G Raghu</name></author><author><name sortKey="Million Rousseau, R" uniqKey="Million Rousseau R">R Million-Rousseau</name></author><author><name sortKey="Morganti, A" uniqKey="Morganti A">A Morganti</name></author><author><name sortKey="Perchenet, L" uniqKey="Perchenet L">L Perchenet</name></author><author><name sortKey="Behr, J" uniqKey="Behr J">J Behr</name></author></analytic></biblStruct></listBibl></div1></back></TEI><pmc article-type="research-article"><pmc-dir>properties open_access</pmc-dir>
<front><journal-meta><journal-id journal-id-type="nlm-ta">PLoS One</journal-id><journal-id journal-id-type="iso-abbrev">PLoS ONE</journal-id><journal-id journal-id-type="publisher-id">plos</journal-id><journal-id journal-id-type="pmc">plosone</journal-id><journal-title-group><journal-title>PLoS ONE</journal-title></journal-title-group><issn pub-type="epub">1932-6203</issn><publisher><publisher-name>Public Library of Science</publisher-name><publisher-loc>San Francisco, CA USA</publisher-loc></publisher></journal-meta><article-meta><article-id pub-id-type="pmid">26630396</article-id><article-id pub-id-type="pmc">4667900</article-id><article-id pub-id-type="doi">10.1371/journal.pone.0141911</article-id><article-id pub-id-type="publisher-id">PONE-D-15-36137</article-id><article-categories><subj-group subj-group-type="heading"><subject>Research Article</subject></subj-group></article-categories><title-group><article-title>Pulmonary Hypertension in Patients with Chronic Fibrosing Idiopathic Interstitial Pneumonias</article-title><alt-title alt-title-type="running-head">PH in Chronic Fibrosing Idiopathic Interstitial Pneumonias</alt-title></title-group><contrib-group><contrib contrib-type="author"><name><surname>Hoeper</surname><given-names>Marius M.</given-names></name><xref ref-type="aff" rid="aff001"><sup>1</sup></xref><xref rid="cor001" ref-type="corresp">*</xref></contrib><contrib contrib-type="author"><name><surname>Behr</surname><given-names>Juergen</given-names></name><xref ref-type="aff" rid="aff002"><sup>2</sup></xref></contrib><contrib contrib-type="author"><name><surname>Held</surname><given-names>Matthias</given-names></name><xref ref-type="aff" rid="aff003"><sup>3</sup></xref></contrib><contrib contrib-type="author"><name><surname>Grunig</surname><given-names>Ekkehard</given-names></name><xref ref-type="aff" rid="aff004"><sup>4</sup></xref></contrib><contrib contrib-type="author"><name><surname>Vizza</surname><given-names>C. Dario</given-names></name><xref ref-type="aff" rid="aff005"><sup>5</sup></xref></contrib><contrib contrib-type="author"><name><surname>Vonk-Noordegraaf</surname><given-names>Anton</given-names></name><xref ref-type="aff" rid="aff006"><sup>6</sup></xref></contrib><contrib contrib-type="author"><name><surname>Lange</surname><given-names>Tobias J.</given-names></name><xref ref-type="aff" rid="aff007"><sup>7</sup></xref></contrib><contrib contrib-type="author"><name><surname>Claussen</surname><given-names>Martin</given-names></name><xref ref-type="aff" rid="aff008"><sup>8</sup></xref></contrib><contrib contrib-type="author"><name><surname>Grohé</surname><given-names>Christian</given-names></name><xref ref-type="aff" rid="aff009"><sup>9</sup></xref></contrib><contrib contrib-type="author"><name><surname>Klose</surname><given-names>Hans</given-names></name><xref ref-type="aff" rid="aff010"><sup>10</sup></xref></contrib><contrib contrib-type="author"><name><surname>Olsson</surname><given-names>Karen M.</given-names></name><xref ref-type="aff" rid="aff001"><sup>1</sup></xref></contrib><contrib contrib-type="author"><name><surname>Zelniker</surname><given-names>Thomas</given-names></name><xref ref-type="aff" rid="aff011"><sup>11</sup></xref></contrib><contrib contrib-type="author"><name><surname>Neurohr</surname><given-names>Claus</given-names></name><xref ref-type="aff" rid="aff002"><sup>2</sup></xref></contrib><contrib contrib-type="author"><name><surname>Distler</surname><given-names>Oliver</given-names></name><xref ref-type="aff" rid="aff012"><sup>12</sup></xref></contrib><contrib contrib-type="author"><name><surname>Wirtz</surname><given-names>Hubert</given-names></name><xref ref-type="aff" rid="aff013"><sup>13</sup></xref></contrib><contrib contrib-type="author"><name><surname>Opitz</surname><given-names>Christian</given-names></name><xref ref-type="aff" rid="aff014"><sup>14</sup></xref></contrib><contrib contrib-type="author"><name><surname>Huscher</surname><given-names>Doerte</given-names></name><xref ref-type="aff" rid="aff015"><sup>15</sup></xref></contrib><contrib contrib-type="author"><name><surname>Pittrow</surname><given-names>David</given-names></name><xref ref-type="aff" rid="aff016"><sup>16</sup></xref></contrib><contrib contrib-type="author"><name><surname>Gibbs</surname><given-names>J. Simon R.</given-names></name><xref ref-type="aff" rid="aff017"><sup>17</sup></xref></contrib></contrib-group><aff id="aff001"><label>1</label><addr-line>Department of Respiratory Medicine and German Center of Lung Research (DZL), Hannover Medical School, Hannover, Germany</addr-line></aff><aff id="aff002"><label>2</label><addr-line>Department of Internal Medicine V, University of Munich, Munich, Germany</addr-line></aff><aff id="aff003"><label>3</label><addr-line>Department of Internal Medicine, Respiratory Medicine and Cardiology, Mission Medical Hospital, Würzburg, Germany</addr-line></aff><aff id="aff004"><label>4</label><addr-line>University Hospital Heidelberg, Heidelberg, Germany</addr-line></aff><aff id="aff005"><label>5</label><addr-line>Department of Cardiovascular and Respiratory Diseases, Sapienza, University of Rome, Rome, Italy</addr-line></aff><aff id="aff006"><label>6</label><addr-line>Department of Pulmonary Diseases, VU University Medical Center, Amsterdam, The Netherlands</addr-line></aff><aff id="aff007"><label>7</label><addr-line>Department of Internal Medicine II, Division of Pneumology, University Medical Center Regensburg, Regensburg, Germany</addr-line></aff><aff id="aff008"><label>8</label><addr-line>LungenClinic, Grosshansdorf, Germany</addr-line></aff><aff id="aff009"><label>9</label><addr-line>Department of Respiratory Medicine, ELK Thorax Centre, Berlin, Germany</addr-line></aff><aff id="aff010"><label>10</label><addr-line>University Medical Center Hamburg-Eppendorf, Center of Oncology, Department of Respiratory Medicine, Hamburg, Germany</addr-line></aff><aff id="aff011"><label>11</label><addr-line>Department of Cardiology, Angiology and Pneumology, University of Heidelberg, Heidelberg, Germany</addr-line></aff><aff id="aff012"><label>12</label><addr-line>Division of Rheumatology, University Hospital Zurich, Zurich, Switzerland</addr-line></aff><aff id="aff013"><label>13</label><addr-line>Department of Respiratory Medicine, University of Leipzig, Leipzig, Germany</addr-line></aff><aff id="aff014"><label>14</label><addr-line>Department of Cardiology, DRK Kliniken Berlin Köpenick, Berlin, Germany</addr-line></aff><aff id="aff015"><label>15</label><addr-line>Department of Rheumatology and Clinical Immunology, Charité University Hospital, and Epidemiology unit, German Rheumatism Research Centre, Berlin, Germany</addr-line></aff><aff id="aff016"><label>16</label><addr-line>Institute for Clinical Pharmacology, Medical Faculty, Technical University, Dresden, Germany</addr-line></aff><aff id="aff017"><label>17</label><addr-line>Department of Cardiology, National Heart & Lung Institute; Imperial College London, London, United Kingdom</addr-line></aff><contrib-group><contrib contrib-type="editor"><name><surname>Kuwana</surname><given-names>Masataka</given-names></name><role>Editor</role><xref ref-type="aff" rid="edit1"></xref></contrib></contrib-group><aff id="edit1"><addr-line>Nippon Medical School Graduate School of Medicine, JAPAN</addr-line></aff><author-notes><fn fn-type="conflict" id="coi001"><p><bold>Competing Interests: </bold>MM Hoeper has received speaker fees and honoraria for consultations from Actelion, Bayer, GSK, and Pfizer. J Behr has received honoraria for lectures and/or consultancy from Actelion, Bayer, Boehringer, Gilead, GSK, InterMune, Lilly, MSD, Novartis, and Pfizer. M Held has received speaker fees and honoraria for consultations from Actelion, Bayer, Boehringer Ingelheim Pharma, Glaxo Smith Kline, Lilly, Janssen, Novartis, Pfizer, Nycomed, Roche and Servier. E Grunig has received honorariums for consultations and/or speaking at conferences from Actelion, Bayer, Gilead, GSK, Lilly, Milteney, Novartis, Pfizer and Rotex Medica and funding for clinical trials by Actelion, Bayer, GSK, Encysive, Lilly and Pfizer. Carmine Dario Vizza has received fees for serving as a speaker, consultant and an advisory board member, from the following companies: Actelion, Dompè, GSK, Italfarmaco, Lilly, Pfizer, United Therapeutics. A Vonk-Noordegraaf reports receiving lecture fees from Actelion, Bayer, GlaxoSmithKline, Lilly and Pfizer, industry advisory board from Actelion and Bayer and serving on steering committees for Actelion, Bayer, GlaxoSmithKline and Pfizer. TJ Lange has received speaker fees, honoraria for consultations and/or research, and educational grants from Actelion, AOP Orphan Pharmaceuticals, Bayer, GSK, Pfizer, and United Therapeutics. M Claussen has received speaker fees and/or honoraria for consultations from Actelion, Bayer and Pfizer. C Grohé has received speaker fees and honoraria for consultations from Actelion, Bayer, Gilead, GSK, Lilly and Pfizer. H Klose has received speaker fees and honoraria for consultations from Actelion, Bayer, GSK, Lilly, Novartis, Pfizer, United Therapeutics and research grants from Actelion. KM Olsson has received speaker fees from Actelion, Bayer and Lilly. T Zelniker has no conflict of interest. C Neurohr has no conflict of interest. O Distler has received speaker fees, honoraria for consultations and/or grant support from 4D Science, Actelion, Active Biotec, Bayer-Schering, Biogen, Biovitrium, BMS, Boehringer, EpiPharm, Ergonex, GSK, Inventiva, Medac, Novartis, Pfizer, Pharmacyclics, Roche/Genentech, Sanofi/Genzyme, Serodapharm, Sinoxa and United BioSource Corporation. H Wirtz has received speaker fees and honoraria for consultations from Bayer, Novartis, GSK, Boehringer Ingelheim Pharma. CF Opitz has no personal conflicts of interest. His institution received speaker fees or honoraria for consultations from Actelion, Bayer, GSK, Lilly, Novartis and Pfizer. D Huscher has received author honoraria from Actelion. D Pittrow has received speaker fees or honoraria for consultations from Actelion, AstraZeneca, Aspen, Bayer, Boehringer Ingelheim, Daiichi Sankyo, Genzyme, Novartis, and Pfizer. JSR Gibbs has received speaker fees and honoraria for consultations from Actelion, Bayer, Gilead, GSK, Lilly, Novartis and Pfizer. These commercial affiliations do not alter the authors adherence to PLOS ONE policies on sharing data and materials.</p></fn><fn fn-type="con" id="contrib001"><p>Conceived and designed the experiments: MMH DH DP JSG. Performed the experiments: MMH JB MH EG CDV AVN TJL MC CG HK KMO TZ CN OD HW CO DH DP JSG. Analyzed the data: DH DP MMH. Wrote the paper: MMH JB MH EG CDV AVN TJL MC CG HK KMO TZ CN OD HW CO DH DP JSG.</p></fn><corresp id="cor001">* E-mail: <email>hoeper.marius@mh-hannover.de</email></corresp></author-notes><pub-date pub-type="epub"><day>2</day><month>12</month><year>2015</year></pub-date><pub-date pub-type="collection"><year>2015</year></pub-date><volume>10</volume><issue>12</issue><elocation-id>e0141911</elocation-id><history><date date-type="received"><day>17</day><month>8</month><year>2015</year></date><date date-type="accepted"><day>14</day><month>10</month><year>2015</year></date></history><permissions><copyright-year>2015</copyright-year><copyright-holder>Hoeper et al</copyright-holder><license xlink:href="http://creativecommons.org/licenses/by/4.0/"><license-p>This is an open access article distributed under the terms of the <ext-link ext-link-type="uri" xlink:href="http://creativecommons.org/licenses/by/4.0/">Creative Commons Attribution License</ext-link>, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited</license-p></license></permissions><self-uri content-type="pdf" xlink:type="simple" xlink:href="pone.0141911.pdf"></self-uri><abstract><sec id="sec001"><title>Background</title><p>Pulmonary hypertension (PH) is a common finding in patients with chronic fibrosing idiopathic interstitial pneumonias (IIP). Little is known about the response to pulmonary vasodilator therapy in this patient population. COMPERA is an international registry that prospectively captures data from patients with various forms of PH receiving pulmonary vasodilator therapies.</p></sec><sec id="sec002"><title>Methods</title><p>We retrieved data from COMPERA to compare patient characteristics, treatment patterns, response to therapy and survival in newly diagnosed patients with idiopathic pulmonary arterial hypertension (IPAH) and PH associated with IIP (PH-IIP).</p></sec><sec id="sec003"><title>Results</title><p>Compared to patients with IPAH (n = 798), patients with PH-IIP (n = 151) were older and predominantly males. Patients with PH-IIP were treated predominantly with phosphodiesterase-5 inhibitors (88% at entry, 87% after 1 year). From baseline to the first follow-up visit, the median improvement in 6MWD was 30 m in patients with IPAH and 24.5 m in patients with PH-IIP (p = 0.457 for the difference between both groups). Improvements in NYHA functional class were observed in 22.4% and 29.5% of these patients, respectively (p = 0.179 for the difference between both groups). Survival rates were significantly worse in PH-IIP than in IPAH (3-year survival 34.0 versus 68.6%; p<0.001). Total lung capacity, NYHA class IV, and mixed-venous oxygen saturation were independent predictors of survival in patients with PH-IIP.</p></sec><sec id="sec004"><title>Conclusions</title><p>Patients with PH-IIP have a dismal prognosis. Our results suggest that pulmonary vasodilator therapy may be associated with short-term functional improvement in some of these patients but it is unclear whether this treatment affects survival.</p></sec><sec id="sec005"><title>Trial Registration</title><p>clinicaltrials.gov <ext-link ext-link-type="uri" xlink:href="https://clinicaltrials.gov/ct2/show/NCT01347216">NCT01347216</ext-link></p></sec></abstract><funding-group><funding-statement>The registry is investigator-initiated and currently supported by unrestricted grants from Actelion, Bayer, GSK, and Pfizer. The funders were not involved at all in this analysis or in preparing the manuscript. The authors without the help of medical writers wrote the paper.</funding-statement></funding-group><counts><fig-count count="2"></fig-count><table-count count="4"></table-count><page-count count="13"></page-count></counts><custom-meta-group><custom-meta id="data-availability"><meta-name>Data Availability</meta-name><meta-value>Due to ethical restrictions, data are available upon request. Although our data are anonymized (Center ID and patient-ID) we are dealing with rare diseases, which is why we are concerned with placing the data into an online repository. These concerns are shared by the lead IRB of this project (Ethics committee, Technical University Dresden, Germany), in particular as the informed consent form that all patients have signed does not include the possibility of an online repository of individual patient data. Interested researchers may submit requests for minimized anonymous dataset by contacting Professor Marius Hoeper (<email>hoeper.marius@mh-hannover.de</email>).</meta-value></custom-meta></custom-meta-group></article-meta><notes><title>Data Availability</title><p>Due to ethical restrictions, data are available upon request. Although our data are anonymized (Center ID and patient-ID) we are dealing with rare diseases, which is why we are concerned with placing the data into an online repository. These concerns are shared by the lead IRB of this project (Ethics committee, Technical University Dresden, Germany), in particular as the informed consent form that all patients have signed does not include the possibility of an online repository of individual patient data. Interested researchers may submit requests for minimized anonymous dataset by contacting Professor Marius Hoeper (<email>hoeper.marius@mh-hannover.de</email>).</p></notes></front></pmc></record>Pour manipuler ce document sous Unix (Dilib)
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