Integrating Clinical Probability into the Diagnostic Approach to Idiopathic Pulmonary Fibrosis: An International Working Group Perspective.
Sara Tomassetti
(1)
,
Claudia Valenzuela
(2)
,
Simon F. L. Walsh
(3)
,
Katerina M. Antoniou
(4)
,
Francesco Bonella
(5)
,
Kevin K. Brown
(6)
,
Harold R. Collard
(7)
,
Tamera J. Corte
(8)
,
Kevin R. Flaherty
(9)
,
Kerri A. Johannson
(10)
,
Martin Kolb
(11)
,
Michael Kreuter
(12)
,
Yoshikazu Inoue
(13)
,
R. Gisli Jenkins
(3)
,
Joyce S. Lee
(14)
,
David A. Lynch
(6)
,
Toby M. Maher
(15, 16)
,
Fernando J. Martinez
(17)
,
Maria Molina-Molina
(18)
,
Jeff L. Myers
(9)
,
S. D. Nathan
(19)
,
Vanerino Poletti
(20)
,
Silvia Quadrelli
(21)
,
Ganesh Raghu
(22)
,
Sujeet K. Rajan
(23)
,
Claudia Ravaglia
(20)
,
Martine Remy
(24, 25)
,
Elisabetta Renzoni
(16)
,
Luca K. Richeldi
(26)
,
Paolo Spagnolo
(27)
,
Marlies Wijsenbeek
(28)
,
Kevin C. Wilson
(29)
,
Wim Wuyts
(30)
,
Athol U. Wells
(3)
,
Christopher J. Ryerson
(31)
1
AOUC -
Azienda Ospedaliero Universitaria Careggi [Firenze] = Careggi University Hospital [Florence, Italy]
2 Hospital Universitario de La Princesa
3 Imperial College London
4 UOC - University of Crete [Heraklion]
5 Ruhrlandklinik University Hospital
6 NJH - National Jewish Health
7 UC San Francisco - University of California [San Francisco]
8 The University of Sydney
9 University of Michigan [Ann Arbor]
10 University of Calgary
11 McMaster University [Hamilton, Ontario]
12 University of Heidelberg, Medical Faculty
13 Kinki University
14 University of Colorado Anschutz [Aurora]
15 USC - University of Southern California
16 Royal Brompton Hospital
17 Cornell University [New York]
18 IDIBELL - Institut d'Investigació Biomèdica de Bellvitge = Bellvitge Biomedical Research Institute
19 IHVI - Inova Heart and Vascular Institute [Falls Church, VA, USA]
20 UNIBO - Alma Mater Studiorum Università di Bologna = University of Bologna
21 Hospital de Clínicas "José de San Martín" [Buenos Aires]
22 University of Washington [Seattle]
23 MU - University of Mumbai
24 CHRU Lille - Centre Hospitalier Régional Universitaire [CHU Lille]
25 METRICS - Evaluation des technologies de santé et des pratiques médicales - ULR 2694
26 Unicatt - Università cattolica del Sacro Cuore = Catholic University of the Sacred Heart [Roma]
27 Unipd - Università degli Studi di Padova = University of Padua
28 Erasmus University Rotterdam
29 BU - Boston University [Boston]
30 University Hospitals Leuven [Leuven]
31 University of British Columbia [Vancouver]
2 Hospital Universitario de La Princesa
3 Imperial College London
4 UOC - University of Crete [Heraklion]
5 Ruhrlandklinik University Hospital
6 NJH - National Jewish Health
7 UC San Francisco - University of California [San Francisco]
8 The University of Sydney
9 University of Michigan [Ann Arbor]
10 University of Calgary
11 McMaster University [Hamilton, Ontario]
12 University of Heidelberg, Medical Faculty
13 Kinki University
14 University of Colorado Anschutz [Aurora]
15 USC - University of Southern California
16 Royal Brompton Hospital
17 Cornell University [New York]
18 IDIBELL - Institut d'Investigació Biomèdica de Bellvitge = Bellvitge Biomedical Research Institute
19 IHVI - Inova Heart and Vascular Institute [Falls Church, VA, USA]
20 UNIBO - Alma Mater Studiorum Università di Bologna = University of Bologna
21 Hospital de Clínicas "José de San Martín" [Buenos Aires]
22 University of Washington [Seattle]
23 MU - University of Mumbai
24 CHRU Lille - Centre Hospitalier Régional Universitaire [CHU Lille]
25 METRICS - Evaluation des technologies de santé et des pratiques médicales - ULR 2694
26 Unicatt - Università cattolica del Sacro Cuore = Catholic University of the Sacred Heart [Roma]
27 Unipd - Università degli Studi di Padova = University of Padua
28 Erasmus University Rotterdam
29 BU - Boston University [Boston]
30 University Hospitals Leuven [Leuven]
31 University of British Columbia [Vancouver]
Résumé
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.