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Health-related quality of life of patients with idiopathic pulmonary fibrosis: a systematic review and meta-analysis

Ingrid A. Cox, Nicolas Borchers Arriagada, Barbara de Graaff, Tamera J. Corte, Ian Glaspole, Stella Lartey, E. Haydn Walters, Andrew J. Palmer
European Respiratory Review 2020 29: 200154; DOI: 10.1183/16000617.0154-2020
Ingrid A. Cox
1Menzies Institute for Medical Research, University of Tasmania, Tasmania, Australia
2Centre of Research Excellence for Pulmonary Fibrosis, Royal Prince Alfred Hospital, Camperdown, Australia
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  • ORCID record for Ingrid A. Cox
Nicolas Borchers Arriagada
1Menzies Institute for Medical Research, University of Tasmania, Tasmania, Australia
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Barbara de Graaff
1Menzies Institute for Medical Research, University of Tasmania, Tasmania, Australia
2Centre of Research Excellence for Pulmonary Fibrosis, Royal Prince Alfred Hospital, Camperdown, Australia
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Tamera J. Corte
2Centre of Research Excellence for Pulmonary Fibrosis, Royal Prince Alfred Hospital, Camperdown, Australia
3Central Clinical School, The University of Sydney, Camperdown, Australia
4Dept of Respiratory and Sleep Medicine, Royal Prince Alfred Hospital, Camperdown, Australia
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Ian Glaspole
2Centre of Research Excellence for Pulmonary Fibrosis, Royal Prince Alfred Hospital, Camperdown, Australia
5Alfred Hospital, Melbourne, Australia
6Monash University, Melbourne, Australia
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Stella Lartey
1Menzies Institute for Medical Research, University of Tasmania, Tasmania, Australia
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E. Haydn Walters
1Menzies Institute for Medical Research, University of Tasmania, Tasmania, Australia
2Centre of Research Excellence for Pulmonary Fibrosis, Royal Prince Alfred Hospital, Camperdown, Australia
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Andrew J. Palmer
1Menzies Institute for Medical Research, University of Tasmania, Tasmania, Australia
2Centre of Research Excellence for Pulmonary Fibrosis, Royal Prince Alfred Hospital, Camperdown, Australia
7Centre for Health Policy, School of Population and Global Health, The University of Melbourne, Melbourne, Australia
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  • For correspondence: andrew.palmer@utas.edu.au
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  • FIGURE 1
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    FIGURE 1

    Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) flow chart illustrating study selection process.

  • FIGURE 2
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    FIGURE 2

    Number of included studies by publication year.

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    FIGURE 3

    Forest plots showing pooled means and subgroup analysis for St George's Respiratory Questionnaire (SGRQ) and Short Form 36 (SF36). QOL: quality of life.

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    FIGURE 4

    Forest plots showing pooled means and sub-group analysis for EuroQoL (EQ5D) and King’s Brief Interstitial Lung Disease questionnaire (KBILD). QOL: quality of life.

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    FIGURE 5

    Summary plots for sensitivity analyses and funnel plot for assessment of publication bias. QOL: quality of life; EQ5D: EuroQoL; SGRQ: St George's Respiratory Questionnaire; SF36: Short Form-36; KBILD: King's Brief Interstitial Lung Disease questionnaire.

Tables

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  • TABLE 1

    Summary of all studies included in the review

    Study [ref.]CountryYearStudy typeClinical diagnosis standards (CDS)CDS yearMeasurement instrumentPopulation characteristics
    Sample sizeMale sexMean ageMean FVC%
    De Vries et al. [77]Netherlands2000Cohort studyNRNRSGRQ
    WHOQOL
    104061.179.6
    Martinez et al. [43]Brazil2000Cross-sectional studyNRNRSF36345952.362.4
    Clark et al. [78]UK2001Prospective observational studyATS/ERS2000SF36506667.780.9
    De Vries et al. [31]Netherlands2001Cross-sectional studyATS/ERS2000WHOQOL413763.5NR
    Baddini et al. [38]Brazil2002Cohort studyATS/ERS2000SF36306058.661.9
    Jastrzebski et al. [41]Poland2005Cross-sectional studyATS/ERS2000SF36166948.344.0
    Nishiyama et al. [79]Japan2005Prospective observational studyATS/ERS2000SGRQ418564.076.6
    Tomioka et al. [80]Japan2005RCTATS/ERS2000SF3622NR70.072.5
    Tzanakis et al. [81]Sweden2005Cross-sectional studyATS/ERS2000SGRQ258066.068.8
    Antoniou et al. [82]Greece2006RCTATS/ERS2000SGRQ508466.971.4
    Tomioka et al. [83]Japan2007Cross-sectional and longitudinal studyATS/ERS2000SF36467069.971.0
    Zimmermann et al. [84]Brazil2007Cross-sectional studyATS/ERS2000SGRQ
    SF36
    20NR61.570.4
    Feltrim et al. [39]Brazil2008Cross-sectional cohort studyNRNRSGRQ75754.1NR
    King et al. [85]Multi-country2008RCT (BUILD-1)ATS/ERS2002SGRQ
    SF36
    1547365.267.8
    Krishnan et al. [86]USA2008Cross-sectional studyATS/ERS2000SF36395467.768.9
    Nishiyama et al. [87]Japan2008RCTATS/ERS2000SGRQ287566.267.5
    Peng et al. [88]China2008Cross-sectional and longitudinal studyATS/ERS2000SGRQ6864.066.0
    Raghu et al. [89]USA2008RCTATS/ERS2000SGRQ
    SF36
    876865.263.9
    Han et al. [90]USA2009Cross-sectional studyATS/ERS2000SGRQ
    SF12
    2216763.062.9
    IPF Clinical Research Network et al. [91]USA2010RCT (STEP-IPF)ATS/ERS2000SGRQ
    EQ5D
    SF36
    1808369.056.8
    Jastrzebski et al. [42]Poland2010Cross-sectional studyNRNRSGRQ
    SF36
    308052.241.2
    Key et al. [92]UK2010Cross-sectional studyATS/ERS2002LCQNR7470.878.5
    Lindell et al. [93]USA2010Mixed method intervention studyATS/ERS2000SF36217666.2NR
    Lutogniewska et al. [51]Poland2010NRNRNRSGRQ
    SF36
    30NRNRNR
    Ozalevli et al. [94]Turkey2010Prospective observational studyATS/ERS2000SF36156762.871.6
    Raghu et al. [95]Multi-country2010RCT (BUILD-1)ATS/ERS2000SGRQ1547365.267.8
    Swigris et al. [74]USA2010Cohort studyATS/ERS2000ATAQ-IPF958269.366.0
    Swigris et al. [76]USA2010RCT (BUILD-1)ATS/ERS2000SGRQ
    SF36
    1007365.166.97
    du Bois et al. [96]Multi-country2011RCTATS/ERS2000SGRQ8227166.072.5
    Elfferich et al. [49]Netherlands2011Cross-sectional studyotherNRWHOQOL496363.182.9
    Jones et al. [97]UK2011Case control studyATS/ERS2002LCQNR6372.080.4
    King et al. [98]Multi-country2011RCT (BUILD-3)ATS/ERS2000SF36
    EQ5D
    5817063.874.5
    Kozu et al. [99]Japan2011Prospective non-randomised open trialATS/ERS2000SF36458267.568.6
    Kozu et al. [100]Japan2011Prospective non-randomised, and uncontrolled studyATS/ERS2000SF36657167.565.3
    Mishra et al. [101]India2011RCTATS/ERS2002SGRQNR10070.761.4
    Rammaert et al. [102]France2011Prospective observational studyATS/ERS2000SGRQ
    SF36
    NR6267.167.0
    Swigris et al. [103]USA2011Prospective observational studyATS/ERS2000SF36218671.573.0
    Verma et al. [45]Canada2011Cross-sectional studyATS/ERS2000SGRQ
    SF36
    1376659.461.7
    Horton et al. [50]USA2012RCTotherNRSGRQ
    CQLQ
    237867.670.4
    Nishiyama et al. [104]Japan2012Retrospective cohort studyATS/ERS2000SGRQ878966.375.0
    Noth et al. [105]USA2012RCT (ACE-IPF)ATS/ERS/JRS/ALAT2011SGRQ
    EQ5D
    SF36
    1457367.058.8
    Raghu et al. [106]USA2012RCT (PANTHER-IPF)ATS/ERS/JRS/ALAT2011SGRQ
    SF36
    1557568.470.7
    Shulgina et al. [107]UK2012RCTATS/ERS2000SGRQ
    EQ5D
    1817271.670.7
    Swigris et al. [108]USA2012RCT (STEP-IPF)ATS/ERS2005SGRQ
    SF36
    1808369.056.8
    Lechtzin et al. [109]USA2013RCTATS/ERS/JRS/ALAT2011SGRQ
    CQLQ
    207567.670.4
    Mermigkis et al. [110]Greece2013Prospective cohort studyATS/ERS/JRS/ALAT2011SF36237868.276.2
    Tzouvelekis et al. [111]Greece2013Non-randomised Clinical TrialATS/ERS/JRS/ALAT2011SGRQ148664.474.2
    Alhamad [112]Saudi Arabia2014Retrospective cohort studyATS/ERS/JRS/ALAT2011SF36585762.965.2
    Gaunaurd et al. [71]USA2014RCTATS/ERS/JRS/ALAT2011SGRQI2168.660.5
    IPF Clinical Research Network et al. [113]USA2014RCT (PANTHER-IPF)ATS/ERS/JRS/ALAT2011SGRQ
    EQ5D
    SF36
    2647867.772.8
    Kozu et al. [114]Japan2014Prospective cross-sectional studyATS/ERS/JRS/ALAT2011SF36657167.565.3
    Lubin et al. [115]USA2014Cohort studyATS/ERS2000SF361027570.070.0
    Morsi et al. [33]Egypt2014Cross-sectional studyNRNRSGRQ362253.046.3
    Richeldi et al. [116]Multi-country2014RCT (INPULSIS-1, INPULSIS-2)ATS/ERS2000SGRQ10617966.879.6
    Rifaat et al. [34]Egypt2014Prospective observational studyATS/ERS/JRS/ALAT2011SGRQ302754.451.9
    Vainshelboim et al. [117]Israel2014RCTATS/ERS/JRS/ALAT2011SGRQ326667.368.2
    Bors et al. [118]USA2015Prospective, cross-sectional studyATS/ERS/JRS/ALAT2011SF36466364.661.7
    Huang et al. [119]China2015RCTATS/ERS/JRS/ALAT2011SGRQ769360.377.3
    Mermigkis et al. [120]Greece2015Prospective cohort studyATS/ERS/JRS/ALAT2011SF3655NR70.3NR
    Nathan et al. [121]Multi-country2015RCT (CAPACITY)ATS/ERS2000SGRQ3387366.574.7
    Raghu et al. [122]Multi-country2015RCTATS/ERS2000SGRQ1268065.067.2
    Vainshelboim et al. [123]Israel2015RCTATS/ERS/JRS/ALAT2011SGRQ286667.368.2
    Atkins et al. [63]UK2016Prospective cohort studyATS/ERS/JRS/ALAT2011SGRQ
    EQ5D
    KBILD
    775976.484.3
    Bahmer et al. [124]Germany2016Prospective cohort studyATS/ERS/JRS/ALAT2011SGRQ
    SF12
    417567.175.4
    Costabel et al. [125]Multi-country2016RCT (INPULSIS-1, INPULSIS-2)ATS/ERS2000SGRQ10617966.879.6
    Ferrara et al. [61]Sweden2016Cross-sectional studyATS/ERS/JRS/ALAT2011KBILD717068.377.5
    Hopkins et al. [47]Canada2016Retrospective population-based studyICDNRHUI2829554NRNR
    Kolb et al. [126]Multi-country2016RCT (INPULSIS-1, INPULSIS-2)ATS/ERS2000SGRQ10817865.578.1
    Kotecha et al. [127]UK2016Cohort studyATS/ERS/JRS/ALAT2011SGRQ755876.483.6
    Richeldi et al. [128]Multi-country2016RCT (TOMORROW-1, INPULSIS-1, INPULSIS-2)ATS/ERS2000SGRQ12317966.579.7
    Taniguchi et al. [129]Multi-country2016RCT (INPULSIS-1, INPULSIS-2)ATS/ERS2000SGRQ3228066.081.2
    Yount et al. [48]USA2016Prospective cohort studyPatient referredNRATAQ-IPF
    PROMIS
    2207061.0NR
    Yu et al. [46]China2016RCTCTSNRSGRQNRNRNRNR
    Arizono et al. [130]Japan2017Prospective observational studyATS/ERS2002SGRQ226470.572.2
    Azuma et al. [131]Japan2017RCT (INPULSIS-1, INPULSIS-2)ATS/ERS2000SGRQ1268168.381.9
    Birring et al. [64]Multi-country2017RCTATS/ERS/JRS/ALAT2011KBILD
    LCQ
    246367.673.0
    Cheng et al. [132]Canada2017Prospective cohort studyATS/ERS/JRS/ALAT2011SGRQ776968.773.2
    Collard et al. [133]Multi-country2017RCT (INPULSIS-1, INPULSIS-2)ATS/ERS2000SGRQ10617966.879.6
    Dowman et al. [52]Australia2017RCTNRNRSGRQI616771.475.9
    Furukawa et al. [134]Japan2017Retrospective cohort studyATS/ERS/JRS/ALAT2011SGRQ1828565.679.7
    Glaspole et al. [53]Australia2017Observational studyNRNRSGRQ4226771.381.0
    Koyama et al. [135]Japan2017Prospective cohort studyATS/ERS/JRS/ALAT2011Bartel Index478975.575.4
    Kreuter et al. [136]Germany2017Observational studyATS/ERS/JRS/ALAT2011SGRQ
    EQ5D
    6237769.667.5
    Lee et al. [137]South Korea2017Prospective cohort studyATS/ERS/JRS/ALAT2011SGRQ1128470.085.0
    Magnani et al. [35]Italy2017Prospective cohort studyATS/ERS/JRS/ALAT2011PGWBI184566.562.4
    Matsuda et al. [138]Japan2017Retrospective cohort studyATS/ERS/JRS/ALAT2011SGRQ1218266.881.1
    Matsuda et al. [139]Japan2017Retrospective cohort studyATS/ERS/JRS/ALAT2011SGRQ
    COPD
    1068567.181.9
    Natalini et al. [140]USA2017Cross-sectional studyATS/ERS/JRS/ALAT2011SF36507870.870.3
    Root et al. [141]USA2017Observational studyATS/ERS/JRS/ALAT2011SF36
    LCQ
    1885665.873.9
    Sokai et al. [142]Japan2017Prospective cohort studyATS/ERS/JRS/ALAT2011SGRQ
    SF36
    528572.086.1
    Swigris et al. [143]Multi-country2017RCT (TOMORROW-1)ATS/ERS2000SGRQ4267565.181.3
    Wapenaar et al. [65]Multi-country2017Prospective cohort studyATS/ERS2000SGRQ
    KBILD
    EQ5D
    1087870.572.5
    Atkins et al. [66]UK2018Prospective observational studyATS/ERS/JRS/ALAT2011KBILD396975.182.7
    Bacci et al. [144]USA2018Cross-sectional qualitative study and secondary quantitative analysis of clinical trial dataATS/ERS/JRS/ALAT2011SGRQ
    EQ5D
    306067.770.2
    Capparelli et al. [70]Argentina2018Cohort study- instrument validationATS/ERS/JRS/ALAT2011SGRQI237871.968.9
    da Fontoura et al. [40]Brazil2018Retrospective cohort studyNRSF36316057.149.0
    Grufstedt et al. [145]Sweden2018Retrospective cohort studyATS/ERS/JRS/ALAT2011SGRQ
    COPD
    876270.184.21
    Kolb et al. [146]Multi-country2018RCT (INSTAGE)ATS/ERS/JRS/ALAT2011SGRQ
    EQ5D
    2737970.267.0
    Mavroudi et al. [147]Greece2018Case control studyATS/ERS2000SF36195869.875.6
    Nishiyama et al. [148]Japan2018Prospective observational studyATS/ERS/JRS/ALAT2011SGRQ317472.373.8
    Nolan et al. [53]UK2018Prospective cohort studyATS/ERS/JRS/ALAT2011KBILD658972.073.2
    Raghu et al. [149]USA2018RCT (WRAP-IPF)ATS/ERS/JRS/ALAT2011SGRQ588269.975.4
    Rajala et al. [150]Finland2018Prospective observational studyATS/ERS/JRS/ALAT2011/2015RAND36927375.078.0
    Rosas et al. [151]USA2018RCTATS/ERS/JRS/ALAT2011SGRQ588167.671.1
    Swigris et al. [152]Multi-country2018RCT (INPULSIS-1, INPULSIS-2)ATS/ERS2000SGRQ
    EQ5D
    10817966.879.6
    Szentes et al. [54]Germany2018Prospective observational studyNRNRKBILD
    EQ5D
    2688663.277.4
    Takeda et al. [153]Japan2018Prospective cohort studyATS/ERS/JRS/ALAT2015COPD258474.480.6
    Vancheri et al. [154]Multi-country2018RCT (INJOURNEY)ATS/ERS/JRS/ALAT2011EQ5D1048668.984.0
    Wuyts et al. [155]Multi-country2018Prospective observational studyATS/ERS/JRS/ALAT2011SGRQ
    EQ5D
    2777769.680.6
    Bosi et al. [156]Italy2019Prospective observational studyATS/ERS/JRS/ALAT2011SGRQ477668.775.5
    Chehere et al. [157]France2019Prospective cohort studyATS/ERS2001SF36197565.075.0
    Eken et al. [158]Turkey2019Cross-sectional studyATS/ERS/JRS/ALAT2011SGRQ407865.586.6
    Justice et al. [159]USA2019RCTATS/ERS/JRS/ALAT2011SGRQ148870.8NR
    Kalafatis et al. [69]Sweden2019Observational studyATS/ERS/JRS/ALAT2011KBILD3487272.070.2
    Kimman et al. [57]Netherlands2019Prospective cohort studyNRNRPESaM
    EQ5D
    1887669NR
    Kreuter et al. [55]Germany2019Observational studyNRNRSGRQ
    EQ5D
    4247768.736.1
    Nolan et al. [56]UK2019Prospective cohort studyNRNRKBILD2096170.071.9
    Pan et al. [44]China2019Cross-sectional studyATS/ERS/JRS/ALAT2011SGRQ
    ATAQ-IPF
    207058.3NR
    Prior et al. [73]Denmark2019Prospective cohort studyATS/ERS/JRS/ALAT2011SGRQI
    SGRQ
    KBILD
    1508172.987.2
    Ryerson et al. [160]Multi-country2019RCT (INPULSIS-1, INPULSIS-2)ATS/ERS2000SGRQ10607966.879.6
    Sinha et al. [36]UK2019Prospective cohort studyNRNRKBILD573362.080.0
    Wallaert et al. [161]France2019Prospective cohort studyATS/ERS/JRS/ALAT2011VSRQ617566.868.6
    Witt et al. [162]Multi-country2019Prospective observational studyATS/ERS/JRS/ALAT2011SF362587367.362.7
    Xu et al. [163]China2019RCT (INPULSIS-1, INPULSIS-2)ATS/ERS2000SGRQ1015464.380.8
    Yuen et al. [164]USA2019RCTATS/ERS/JRS/ALAT2011SGRQ206569.866.0
    Bloem et al. [37]Netherlands2020Cross-sectional studyATS/ERS/JRS/ALAT2011EQ5D
    QoL-RIQ
    594573.083.2
    Case et al. [165]USA2020Retrospective cohort studyATS/ERS/JRS/ALAT2011SGRQ
    EQ5D
    SF12
    6627570.069.9
    Janssen et al. [166]USA2020RCTATS/ERS/JRS/ALAT2011SGRQ227190.973.4
    Kreuter et al. [167]Multi-country2020RCT (INPULSIS-1, INPULSIS-2)ATS/ERS2000SGRQ10617966.879.6
    Moor et al. [67]Netherlands2020RCTATS/ERS/JRS/ALAT2018PESaM
    EQ5D
    KBILD
    909171.080.1
    O'Brien et al. [168]USA2020Cross-sectional studyATS/ERS/JRS/ALAT2011SGRQ
    EQ5D
    SF12
    8597470.069.7
    Prior et al. [72]Denmark2020Prospective cohort studyATS/ERS/JRS/ALAT2011SGRQI
    SGRQ
    KBILD
    1508172.987.2
    Richeldi et al. [169]Multi-country2020RCT (INPULSIS-1, INPULSIS-2, INSTAGE)ATS/ERS
    ATS/ERS/JRS/ALAT
    2000
    2011
    SGRQ11977967.178.0
    Tzouvelekis et al. [68]Greece2020Prospective cohort studyATS/ERS/JRS/ALAT2011SGRQ
    KBILD
    988370.877.0

    All means are calculated pooled means. ACE-IPF: Anti-Coagulant Effectiveness in Idiopathic Pulmonary Fibrosis; ATS/ERS: American Thoracic Society/European Respiratory Society; ATS/ERS/JRS/ALAT: American Thoracic Society/European Respiratory Society/Japanese Respiratory Society/Latin American Thoracic Society; ATAQ-IPF: A Tool to Assess Quality of life in IPF; BUILD: Bosentan Use in Interstitial Lung Disease; CQLQ: The Cough Quality of Life Questionnaire; CTS: Chinese Thoracic Society; EQ5D: EuroQol; FVC: Forced vital capacity; HUI2: Health Utilities Index Mark 2; IPF: idiopathic pulmonary fibrosis; K-BILD: King's Brief Interstitial Lung Disease; LCQ: Leicester Cough Questionnaire; NR: not reported; PANTHER Prednisone, Azathioprine, and N-acetylcysteine in Participants With IPF; PESaM: Patient Experiences and Satisfaction with Medications Questionnaire; PGWBI: The Psychological General Well-Being Index; PROMIS: Participant Reported Outcomes Measurement Information System; QoL-RIQ: Quality-of-Life for Respiratory Illness; RAND36: Research and Development 36 item health survey; RCT: Randomised controlled trial; SF12: Short Form-12; SF36 Short Form-36; SGRQ: St George's Questionnaire; SGRQI: IPF-specific version of the SGRQ; STEP-IPF: Sildenafil Trial Of Exercise Performance In Idiopathic Pulmonary Fibrosis; VSRQ: Visual Simplified Respiratory Questionnaire; WHOQoL: WHO Quality of Life Questionnaire; WRAP-IPF: Treatment of IPF With Laparoscopic Anti-Reflux Surgery.

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    Health-related quality of life of patients with idiopathic pulmonary fibrosis: a systematic review and meta-analysis
    Ingrid A. Cox, Nicolas Borchers Arriagada, Barbara de Graaff, Tamera J. Corte, Ian Glaspole, Stella Lartey, E. Haydn Walters, Andrew J. Palmer
    European Respiratory Review Dec 2020, 29 (158) 200154; DOI: 10.1183/16000617.0154-2020

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    Health-related quality of life of patients with idiopathic pulmonary fibrosis: a systematic review and meta-analysis
    Ingrid A. Cox, Nicolas Borchers Arriagada, Barbara de Graaff, Tamera J. Corte, Ian Glaspole, Stella Lartey, E. Haydn Walters, Andrew J. Palmer
    European Respiratory Review Dec 2020, 29 (158) 200154; DOI: 10.1183/16000617.0154-2020
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