Table of Contents

Original Articles

• Pulmonary vascular disease

  • Open Access

    The ADAMTS13–VWF axis is dysregulated in chronic thromboembolic pulmonary hypertension

    Michael Newnham, Kieron South, Marta Bleda, William R. Auger, Joan A. Barberà, Harm Bogaard, Katherine Bunclark, John E. Cannon, Marion Delcroix, Charaka Hadinnapola, Luke S. Howard, David Jenkins, Eckhard Mayer, Choo Ng, Christopher J. Rhodes, Nicholas Screaton, Karen Sheares, Michael A. Simpson, Mark Southwood, Li Su, Dolores Taboada, Matthew Traylor, Richard C. Trembath, Sofia S. Villar, Martin R. Wilkins, John Wharton, Stefan Gräf, Joanna Pepke-Zaba, Michael Laffan, David A. Lane, Nicholas W. Morrell, Mark Toshner

    European Respiratory Journal 53 (3) 1801805; DOI: 10.1183/13993003.01805-2018 Published 28 March 2019
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    Widening the landscape of heritable pulmonary hypertension mutations in paediatric and adult cases

    Mélanie Eyries, David Montani, Sophie Nadaud, Barbara Girerd, Marilyne Levy, Arnaud Bourdin, Romain Trésorier, Ari Chaouat, Vincent Cottin, Céline Sanfiorenzo, Grégoire Prevot, Martine Reynaud-Gaubert, Claire Dromer, Ali Houeijeh, Karine Nguyen, Florence Coulet, Damien Bonnet, Marc Humbert, Florent Soubrier

    European Respiratory Journal 53 (3) 1801371; DOI: 10.1183/13993003.01371-2018 Published 14 March 2019
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    Germline BMP9 mutation causes idiopathic pulmonary arterial hypertension

    Xiao-Jian Wang, Tian-Yu Lian, Xin Jiang, Shao-Fei Liu, Su-Qi Li, Rong Jiang, Wen-Hui Wu, Jue Ye, Chun-Yan Cheng, Yao Du, Xi-Qi Xu, Yan Wu, Fu-Hua Peng, Kai Sun, Yi-Min Mao, Huan Yu, Chen Liang, John Y-J. Shyy, Shu-Yang Zhang, Xue Zhang, Zhi-Cheng Jing

    European Respiratory Journal 53 (3) 1801609; DOI: 10.1183/13993003.01609-2018 Published 14 March 2019

• Cystic fibrosis

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    Cluster and CART analyses identify large subgroups of adults with cystic fibrosis at low risk of 10-year death

    Pierre-Régis Burgel, Lydie Lemonnier, Clémence Dehillotte, Jenna Sykes, Sanja Stanojevic, Anne L. Stephenson, Jean-Louis Paillasseur

    European Respiratory Journal 53 (3) 1801943; DOI: 10.1183/13993003.01943-2018 Published 14 March 2019

• Cystic fibrosis and basic science

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    Targeting of cathepsin S reduces cystic fibrosis-like lung disease

    Donna M. Small, Ryan R. Brown, Declan F. Doherty, Anthony Abladey, Zhe Zhou-Suckow, Rebecca J. Delaney, Lauren Kerrigan, Caoifa M. Dougan, Keren S. Borensztajn, Leslie Holsinger, Robert Booth, Christopher J. Scott, Guillermo López-Campos, J. Stuart Elborn, Marcus A. Mall, Sinéad Weldon, Clifford C. Taggart

    European Respiratory Journal 53 (3) 1801523; DOI: 10.1183/13993003.01523-2018 Published 28 March 2019

• Basic science

  • Open Access

    Neutrophils disturb pulmonary microcirculation in sepsis-induced acute lung injury

    Inwon Park, Mingyo Kim, Kibaek Choe, Eunjoo Song, Howon Seo, Yoonha Hwang, Jinhyo Ahn, Seung-Hyo Lee, Jae Hyuk Lee, You Hwan Jo, Kyuseok Kim, Gou Young Koh, Pilhan Kim

    European Respiratory Journal 53 (3) 1800786; DOI: 10.1183/13993003.00786-2018 Published 28 March 2019

    • Supplementary videos
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    Fibrillin-2 is a key mediator of smooth muscle extracellular matrix homeostasis during mouse tracheal tubulogenesis

    Wenguang Yin, Hyun-Taek Kim, ShengPeng Wang, Felix Gunawan, Rui Li, Carmen Buettner, Beate Grohmann, Gerhard Sengle, Debora Sinner, Stefan Offermanns, Didier Y.R. Stainier

    European Respiratory Journal 53 (3) 1800840; DOI: 10.1183/13993003.00840-2018 Published 7 March 2019

• Lung structure and function

  • Open Access

    Relative and absolute lung function change in a general population aged 60–102 years

    Johannes Luoto, Mats Pihlsgård, Per Wollmer, Sölve Elmståhl

    European Respiratory Journal 53 (3) 1701812; DOI: 10.1183/13993003.01812-2017 Published 14 March 2019

• Lung cancer and imaging

  • Open Access

    Predicting EGFR mutation status in lung adenocarcinoma on computed tomography image using deep learning

    Shuo Wang, Jingyun Shi, Zhaoxiang Ye, Di Dong, Dongdong Yu, Mu Zhou, Ying Liu, Olivier Gevaert, Kun Wang, Yongbei Zhu, Hongyu Zhou, Zhenyu Liu, Jie Tian

    European Respiratory Journal 53 (3) 1800986; DOI: 10.1183/13993003.00986-2018 Published 28 March 2019

• Lung cancer

  • Open Access

    Which definition of a central tumour is more predictive of occult mediastinal metastasis in nonsmall cell lung cancer patients with radiological N0 disease?

    Sun Hye Shin, Dong Young Jeong, Kyung Soo Lee, Jong Ho Cho, Yong Soo Choi, Kyungjong Lee, Sang-Won Um, Hojoong Kim, Byeong-Ho Jeong

    European Respiratory Journal 53 (3) 1801508; DOI: 10.1183/13993003.01508-2018 Published 18 March 2019
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    Role of anlotinib-induced CCL2 decrease in anti-angiogenesis and response prediction for nonsmall cell lung cancer therapy

    Jun Lu, Hua Zhong, Tianqing Chu, Xueyan Zhang, Rong Li, Jiayuan Sun, Runbo Zhong, Yuqin Yang, Mohammad Shah Alam, Yuqing Lou, Jianlin Xu, Yanwei Zhang, Jun Wu, Xiaowei Li, Xiaodong Zhao, Kai Li, Liming Lu, Baohui Han

    European Respiratory Journal 53 (3) 1801562; DOI: 10.1183/13993003.01562-2018 Published 7 March 2019

• COPD

  • Open Access

    Dual bronchodilation with tiotropium/olodaterol further reduces activity-related breathlessness versus tiotropium alone in COPD

    François Maltais, Joseph-Leon Aumann, Anne-Marie Kirsten, Éric Nadreau, Hemani Macesic, Xidong Jin, Alan Hamilton, Denis E. O'Donnell

    European Respiratory Journal 53 (3) 1802049; DOI: 10.1183/13993003.02049-2018 Published 28 March 2019
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    Serum levels of hyaluronic acid are associated with COPD severity and predict survival

    Eleni Papakonstantinou, Ioannis Bonovolias, Michael Roth, Michael Tamm, Desiree Schumann, Florent Baty, Renaud Louis, Branislava Milenkovic, Wim Boersma, Bram Stieltjes, Konstantinos Kostikas, Francesco Blasi, Joachim G. Aerts, Gernot G.U. Rohde, Alicia Lacoma, Antoni Torres, Tobias Welte, Daiana Stolz

    European Respiratory Journal 53 (3) 1801183; DOI: 10.1183/13993003.01183-2018 Published 7 March 2019

• Infection

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    Patient-reported outcome measures in the recovery of adults hospitalised with community-acquired pneumonia: a systematic review

    Harry J. Pick, Charlotte E. Bolton, Wei Shen Lim, Tricia M. McKeever

    European Respiratory Journal 53 (3) 1802165; DOI: 10.1183/13993003.02165-2018 Published 18 March 2019

• Infection and tuberculosis

  • Open Access

    Chronic pulmonary aspergillosis commonly complicates treated pulmonary tuberculosis with residual cavitation

    Iain D. Page, Rosemary Byanyima, Sharath Hosmane, Nathan Onyachi, Cyprian Opira, Malcolm Richardson, Richard Sawyer, Anna Sharman, David W. Denning

    European Respiratory Journal 53 (3) 1801184; DOI: 10.1183/13993003.01184-2018 Published 18 March 2019

• Tuberculosis

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    Introducing molecular testing of pyrazinamide susceptibility improves multidrug-resistant tuberculosis treatment outcomes: a prospective cohort study

    Feng Sun, Yang Li, Yu Chen, Wenlong Guan, Xiangao Jiang, Xiaomeng Wang, Pengfei Ren, Junlian Li, Jichan Shi, Guiqing He, Meiying Wu, Peijun Tang, Fei Wang, Yunfeng Sheng, Fuli Huang, Zumo Zhou, Heqing Huang, Liang Hong, Qihui Liu, Ying Zhang, Wenhong Zhang

    European Respiratory Journal 53 (3) 1801770; DOI: 10.1183/13993003.01770-2018 Published 14 March 2019
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    Adjunctive vitamin D in tuberculosis treatment: meta-analysis of individual participant data

    David A. Jolliffe, Davaasambuu Ganmaa, Christian Wejse, Rubhana Raqib, M. Ahsanul Haq, Nawal Salahuddin, Peter K. Daley, Anna P. Ralph, Thomas R. Ziegler, Adrian R. Martineau

    European Respiratory Journal 53 (3) 1802003; DOI: 10.1183/13993003.02003-2018 Published 7 March 2019

• Interstitial lung diseases

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    A randomised, placebo-controlled study of omipalisib (PI3K/mTOR) in idiopathic pulmonary fibrosis

    Pauline T. Lukey, Stephen A. Harrison, Shuying Yang, Yim Man, Beverley F. Holman, Alaleh Rashidnasab, Gabrielle Azzopardi, Michael Grayer, Juliet K. Simpson, Philippe Bareille, Lyn Paul, Hannah V. Woodcock, Richard Toshner, Peter Saunders, Philip L. Molyneaux, Kris Thielemans, Frederick J. Wilson, Paul F. Mercer, Rachel C. Chambers, Ashley M. Groves, William A. Fahy, Richard P. Marshall, Toby M. Maher

    European Respiratory Journal 53 (3) 1801992; DOI: 10.1183/13993003.01992-2018 Published 18 March 2019
  • Open Access

    Phase 2 clinical trial of PBI-4050 in patients with idiopathic pulmonary fibrosis

    Nasreen Khalil, Helene Manganas, Christopher J. Ryerson, Shane Shapera, Andre M. Cantin, Paul Hernandez, Eric E. Turcotte, Joseph M. Parker, John E. Moran, Gary R. Albert, Renata Sawtell, Aline Hagerimana, Pierre Laurin, Lyne Gagnon, Frank Cesari, Martin Kolb

    European Respiratory Journal 53 (3) 1800663; DOI: 10.1183/13993003.00663-2018 Published 18 March 2019
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    The added value of comorbidities in predicting survival in idiopathic pulmonary fibrosis: a multicentre observational study

    Sebastiano Emanuele Torrisi, Brett Ley, Michael Kreuter, Marlies Wijsenbeek, Eric Vittinghoff, Harold R. Collard, Carlo Vancheri

    European Respiratory Journal 53 (3) 1801587; DOI: 10.1183/13993003.01587-2018 Published 7 March 2019