Simon L F Walsh1, David J Lederer2,3, Christopher J Ryerson4, Martin Kolb2,5, Toby M Maher1,6, Richard Nusser7, Venerino Poletti8,9, Luca Richeldi10, Carlo Vancheri11, Margaret L Wilsher12, Katerina M Antoniou13, Juergen Behr14, Elisabeth Bendstrup9, Kevin K Brown15, Tamera J Corte16, Vincent Cottin17, Bruno Crestani18, Kevin R Flaherty19, Ian N Glaspole20, Jan Grutters21, Yoshikazu Inoue22, Yasuhiro Kondoh23, Michael Kreuter24, Kerri A Johannson25, Brett Ley26, Fernando J Martinez27, Maria Molina-Molina28, Antonio Morais29, Hilario Nunes30, Ganesh Raghu31, Moises Selman32, Paolo Spagnolo33, Hiroyuki Taniguchi23, Sara Tomassetti8, Dominique Valeyre30, Marlies Wijsenbeek34, Wim A Wuyts35, Athol U Wells36. 1. National Heart and Lung Institute, Imperial College, London, United Kingdom. 2. Department of Medicine and. 3. Department of Epidemiology, Columbia University Medical Center, New York, New York. 4. Department of Medicine, University of British Columbia, Vancouver, British Columbia, Canada. 5. Department of Pathology and Molecular Medicine, Firestone Institute for Respiratory Health, McMaster University, Hamilton, Ontario, Canada. 6. National Institute of Health Research Respiratory Clinical Research Facility and. 7. Department of Respiratory Medicine, Summit Hospital, Oakland, California. 8. Department of Diseases of the Thorax, Ospedale G. B. Morgagni, Forlì, Italy. 9. Department of Respiratory Diseases and Allergy, Aarhus University Hospital, Aarhus, Denmark. 10. Fondazione Policlinico A. Gemelli, Istituto di Ricovero e Carattere Scientifico, Università Cattolica del Sacro Cuore, Rome, Italy. 11. Department of Clinical and Experimental Medicine, Regional Referral Centre for Rare Lung Diseases, University-Hospital "Policlinico" Vittorio Emanuele, University of Catania, Catania, Italy. 12. Auckland District Health Board, University of Auckland, Auckland, New Zealand. 13. Department of Respiratory Medicine, Faculty of Medicine, University of Crete, Heraklion, Greece. 14. Department of Medicine V, University of Munich and Asklepios Fachkliniken Gauting, Comprehensive Pneumology Center, member of the German Center for Lung Research [DZL], Munich, Germany. 15. National Jewish Health, Denver, Colorado. 16. Department of Respiratory Medicine, Royal Prince Alfred Hospital, University of Sydney, Sydney, Australia. 17. National Reference Center for Rare Pulmonary Diseases, Louis Pradel Hospital, UMR 754, Claude Bernard University Lyon 1, Lyon, France. 18. APHP, Hopital Bichat, Service de Pneumologie A, Université Paris Diderot, Paris, France. 19. Division of Pulmonary and Critical Care Medicine, University of Michigan, Ann Arbor, Michigan. 20. Alfred Health-Allergy, Immunology, and Respiratory Medicine, the Alfred Hospital, Melbourne, Australia. 21. Division of Heart and Lungs, ILD Center of Excellence, St. Antonius Hospital, University Medical Centre Utrecht, Utrecht, the Netherlands. 22. Clinical Research Center, National Hospital Organization Kinki-Chuo Chest Medical Center, Osaka, Japan. 23. Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Japan. 24. Center for Interstitial and Rare Lung Diseases, Pneumology and Respiratory Critical Care Medicine, Thoraxklinik, University of Heidelberg and Translational Lung Research Center Heidelberg, member of the DZL, Heidelberg, Germany. 25. University of Calgary, Calgary, Alberta, Canada. 26. Kaiser Permanente San Francisco, San Francisco, California. 27. Weill Cornell Medicine, New York, New York. 28. University Hospital of Bellvitge, IDIBELL-CIBERES, Barcelona, Spain. 29. Pulmonology, Faculdade de Medicina do Porto, Centro Hospitalar São João, Oporto, Portugal. 30. INSERM UMR 1272, Paris 13 University, Sorbonne Paris Cité, Service de Pneumologie, Hopital Avicenne, Bobigny, France. 31. Center for Interstitial Lung Disease, University of Washington, Seattle, Washington. 32. Instituto Nacional de Enfermedades Respiratorias "Ismael Cosio Villegas," Mexico City, Mexico. 33. Respiratory Disease Unit, Department of Cardiac, Thoracic, and Vascular Sciences, University of Padova, Padova, Italy. 34. Department of Pulmonary Diseases, Erasmus Medical Center, University Hospital Rotterdam, Rotterdam, the Netherlands; and. 35. Department of Pulmonary Medicine, Unit for Interstitial Lung Diseases, University Hospitals Leuven, Leuven, Belgium. 36. Interstitial Lung Disease Unit, Royal Brompton and Harefield Foundation Trust, London, United Kingdom.
Abstract
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.
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.
Entities:
Keywords:
antifibrotic therapy; clinical practice guidelines; idiopathic pulmonary fibrosis; surgical lung biopsy; working diagnosis
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