Diagnostic likelihood thresholds that define a working diagnosis of idiopathic pulmonary fibrosis

Research output: Contribution to journal/Conference contribution in journal/Contribution to newspaperJournal articleResearchpeer-review

  • Simon L.F. Walsh, Imperial College London
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  • David J. Lederer, Columbia University
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  • Christopher J. Ryerson, The University of British Columbia
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  • Martin Kolb, McMaster University
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  • Toby M. Maher, Imperial College London
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  • Richard Nusser, Summit Hospital
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  • Venerino Poletti
  • Luca Richeldi, Fondazione Policlinico Universitario Agostino Gemelli IRCCS Università Cattolica del Sacro Cuore
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  • Carlo Vancheri, Università di Catania
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  • Margaret L. Wilsher, The University of Auckland
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  • Katerina M. Antoniou, University of Crete
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  • Juergen Behr, Ludwig-Maximilians-University München
  • ,
  • Elisabeth Bendstrup
  • Kevin K. Brown, University of Colorado Denver
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  • Tamera J. Corte, Royal Prince Alfred Hospital
  • ,
  • Vincent Cottin, Claude Bernard University Lyon 1
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  • Bruno Crestani, Universite Paris 7
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  • Kevin R. Flaherty, University of Michigan, Ann Arbor, Michigan.
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  • Ian N. Glaspole, Alfred Hospital
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  • Jan Grutters, Utrecht University
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  • Yoshikazu Inoue, National Hospital Organization, Japan
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  • Yasuhiro Kondoh, Tosei General Hospital
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  • Michael Kreuter, Universität Heidelberg
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  • Kerri A. Johannson, University of Calgary
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  • Brett Ley, Kaiser Permanente
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  • Fernando J. Martinez, Weill Cornell Medicine
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  • Maria Molina-Molina, Hospital Universitari de Bellvitge
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  • Antonio Morais, Sao Joao Hospital
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  • Hilario Nunes, Inserm
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  • Ganesh Raghu, University of Washington, Seattle
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  • Moises Selman, National Institute of Respiratory Diseases
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  • Paolo Spagnolo, University of Padova
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  • Hiroyuki Taniguchi, Tosei General Hospital
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  • Sara Tomassetti, Ospedale Gb Morgagni
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  • Dominique Valeyre, Inserm
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  • Marlies Wijsenbeek, Erasmus University Medical Center
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  • Wim A. Wuyts, KU Leuven
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  • Athol U. Wells, Imperial College London

Rationale: The level of diagnostic likelihood at which physicians prescribe antifibrotic therapy without requesting surgical lung biopsy (SLB) in patients suspected of idiopathic pulmonary fibrosis (IPF) is unknown. Objectives: To determine how often physicians advocate SLB in patient subgroups defined by IPF likelihood and risk associated with SLB, and to identify the level of diagnostic likelihood at which physicians prescribe antifibrotic therapy with requesting SLB. Methods: An international cohort of respiratory physicians evaluated 60 cases of interstitial lung disease, giving: 1) differential diagnoses with diagnostic likelihood; 2) a decision on the need for SLB; and 3) initial management. Diagnoses were stratified according to diagnostic likelihood bands described by Ryerson and colleagues. Measurements and Main Results: A total of 404 physicians evaluated the 60 cases (24,240 physician-patient evaluations). IPF was part of the differential diagnosis in 9,958/24,240 (41.1%) of all physician-patient evaluations. SLB was requested in 8.1%, 29.6%, and 48.4% of definite, provisional high-confidence and provisional low-confidence diagnoses of IPF, respectively. In 63.0% of provisional high-confidence IPF diagnoses, antifibrotic therapy was prescribed without requesting SLB. No significant mortality difference was observed between cases given a definite diagnosis of IPF (90-100% diagnostic likelihood) and cases given a provisional high-confidence IPF diagnosis (hazard ratio, 0.97; P = 0.65; 95% confidence interval, 0.90-1.04). Conclusions: Most respiratory physicians prescribe antifibrotic therapy without requesting an SLB if a provisional high-confidence diagnosis or "working diagnosis" of IPF can be made (likelihood>70%). SLB is recommended in only a minority of patients with suspected, but not definite, IPF.

Original languageEnglish
JournalAmerican Journal of Respiratory and Critical Care Medicine
Volume200
Issue9
Pages (from-to)1146-1153
Number of pages8
ISSN1073-449X
DOIs
Publication statusPublished - 2019

    Research areas

  • Antifibrotic therapy, Clinical practice guidelines, Idiopathic pulmonary fibrosis, Surgical lung biopsy, Working diagnosis

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