Vincent Cottin1, Sara Tomassetti2, Claudia Valenzuela3, Simon L F Walsh4, Katerina M Antoniou5, Francesco Bonella6, Kevin K Brown7, Harold R Collard8, Tamera J Corte9, Kevin R Flaherty10, Kerri A Johannson11,12, Martin Kolb13, Michael Kreuter14,15, Yoshikazu Inoue16, R Gisli Jenkins4, Joyce S Lee17, David A Lynch7, Toby M Maher18,19, Fernando J Martinez20, Maria Molina-Molina21, Jeff L Myers22, Steven D Nathan23, Venerino Poletti24, Silvia Quadrelli25, Ganesh Raghu26, Sujeet K Rajan27, Claudia Ravaglia28, Martine Remy-Jardin29, Elisabetta Renzoni19, Luca K Richeldi30, Paolo Spagnolo31, Lauren Troy32,33, Marlies Wijsenbeek34, Kevin C Wilson35, Wim Wuyts36, Athol U Wells4, Christopher J Ryerson37. 1. National Reference Center for Rare Pulmonary Diseases, Louis Pradel Hospital, Hospices Civils de Lyon, University of Lyon, Institut National de Recherche pour l'Agriculture et l'Environnement (INRAE), European Reference Network (ERN-LUNG), Lyon, France. 2. Department of Experimental and Clinical Medicine, Careggi University Hospital, Florence, Italy. 3. Pulmonology Department, Hospital Universitario de la Princesa, Departamento Medicina, Universidad Autónoma de Madrid, Madrid, Spain. 4. Imperial College London, London, United Kingdom. 5. University of Heraklion, Heraklion, Crete, Greece. 6. Center for Interstitial and Rare Lung Diseases, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany. 7. National Jewish Health Medical and Research Center, Denver, Colorado. 8. Department of Medicine, University of California San Francisco, San Francisco, California. 9. Royal Prince Alfred Hospital and University of Sydney, Sydney, New South Wales, Australia. 10. Division of Pulmonary and Critical Care Medicine and. 11. Department of Medicine and. 12. Department of Community Health Sciences, University of Calgary, Calgary, Alberta, Canada. 13. Department of Medicine, Firestone Institute for Respiratory Health, St. Joseph's Healthcare, McMaster University, Hamilton, Ontario, Canada. 14. Center for Interstitial and Rare Lung Diseases, Department of Pneumology, Thoraxklinik, University Hospital Heidelberg, Heidelberg, Germany. 15. German Center for Lung Research, Heidelberg, Germany. 16. Kinki-Chuo Chest Medical Center, Clinical Research Center, National Hospital Organization, Osaka, Japan. 17. School of Medicine, Anschutz Medical Campus, University of Colorado Denver, Aurora, Colorado. 18. Division of Pulmonary, Critical Care and Sleep Medicine, Keck School of Medicine, University of Southern California, Los Angeles, California. 19. Interstitial Lung Disease Unit, Brompton Hospital, London, United Kingdom. 20. Cornell Medical College, New York, New York. 21. Interstitial Lung Disease Unit, Respiratory Department, University Hospital of Bellvitge, IDIBELL, CIBERES, Barcelona, Spain. 22. Division of Anatomic Pathology, University of Michigan, Ann Arbor, Michigan. 23. Advanced Lung Disease and Transplant Program, Inova Fairfax Hospital, Falls Church, Virginia. 24. Department of Diseases of the Thorax and. 25. Buenos Aires British Hospital, Buenos Aires, Argentina. 26. Center for Interstitial Lung Diseases, Department of Medicine, Department of Laboratory Medicine and Pathology, University of Washington, Seattle, Washington. 27. Bombay Hospital Institute of Medical Sciences and Bhatia Hospital, Mumbai, India. 28. Department of Thoracic Diseases, G.B. Morgagni Hospital, University of Bologna, Forlì, Italy. 29. Thoracic Imaging, Calmette Hospital, Lille, France. 30. UOC Pneumologia, Universita Cattolica del Sacro Cuore Sede di Roma, Rome, Italy. 31. Respiratory Disease Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padova, Padova, Italy. 32. Department of Respiratory Medicine, Royal Prince Alfred Hospital, Sydney, Australia. 33. Sydney Medical School, University of Sydney, Sydney, Australia. 34. Erasmus University Rotterdam, Rotterdam, the Netherlands. 35. Division of Allergy, Pulmonary, Critical Care, and Sleep Medicine, School of Medicine, Boston University, Boston, Massachusetts. 36. Department of Respiratory Medicine, K U Leuven, Leuven, Belgium; and. 37. University of British Columbia Faculty of Medicine, Vancouver, British Columbia, Canada.
Abstract
Background: When considering the diagnosis of idiopathic pulmonary fibrosis (IPF), experienced clinicians integrate clinical features that help to differentiate IPF from other fibrosing interstitial lung diseases, thus generating a "pre-test" probability of IPF. The aim of this international working group perspective was to summarize these features using a tabulated approach similar to chest HRCT and histopathologic patterns reported in the international guidelines for the diagnosis of IPF, and to help formally incorporate these clinical likelihoods into diagnostic reasoning to facilitate the diagnosis of IPF. Methods: The committee group identified factors that influence the clinical likelihood of a diagnosis of IPF, which was categorized as a pre-test clinical probability of IPF into "high" (70-100%), "intermediate" (30-70%), or "low" (0-30%). After integration of radiological and histopathological features, the post-test probability of diagnosis was categorized into "definite" (90-100%), "high confidence" (70-89%), "low confidence" (51-69%), or "low" (0-50%) probability of IPF. Findings: A conceptual Bayesian framework was created, integrating the clinical likelihood of IPF ("pre-test probability of IPF") with the HRCT pattern, the histopathology pattern when available, and/or the pattern of observed disease behavior, into a "post-test probability of IPF." The diagnostic probability of IPF was expressed using an adapted diagnostic ontology for fibrotic interstitial lung diseases. Interpretation: The present approach will help incorporate the clinical judgment into the diagnosis of IPF, thus facilitating the application of IPF diagnostic guidelines and, ultimately improving diagnostic confidence and reducing the need for invasive diagnostic techniques.
Background: When considering the diagnosis of idiopathic pulmonary fibrosis (IPF), experienced clinicians integrate clinical features that help to differentiate IPF from other fibrosing interstitial lung diseases, thus generating a "pre-test" probability of IPF. The aim of this international working group perspective was to summarize these features using a tabulated approach similar to chest HRCT and histopathologic patterns reported in the international guidelines for the diagnosis of IPF, and to help formally incorporate these clinical likelihoods into diagnostic reasoning to facilitate the diagnosis of IPF. Methods: The committee group identified factors that influence the clinical likelihood of a diagnosis of IPF, which was categorized as a pre-test clinical probability of IPF into "high" (70-100%), "intermediate" (30-70%), or "low" (0-30%). After integration of radiological and histopathological features, the post-test probability of diagnosis was categorized into "definite" (90-100%), "high confidence" (70-89%), "low confidence" (51-69%), or "low" (0-50%) probability of IPF. Findings: A conceptual Bayesian framework was created, integrating the clinical likelihood of IPF ("pre-test probability of IPF") with the HRCT pattern, the histopathology pattern when available, and/or the pattern of observed disease behavior, into a "post-test probability of IPF." The diagnostic probability of IPF was expressed using an adapted diagnostic ontology for fibrotic interstitial lung diseases. Interpretation: The present approach will help incorporate the clinical judgment into the diagnosis of IPF, thus facilitating the application of IPF diagnostic guidelines and, ultimately improving diagnostic confidence and reducing the need for invasive diagnostic techniques.
Authors: Paolo Cameli; Valerio Alonzi; Miriana d'Alessandro; Laura Bergantini; Elena Pordon; Marco Guerrieri; Rosa Metella Refini; Piersante Sestini; Elena Bargagli Journal: Biomedicines Date: 2022-08-15