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

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Title: Diagnostic likelihood thresholds that define a working diagnosis of idiopathic pulmonary fibrosis
Authors: Walsh, SLF
Lederer, DJ
Ryerson, CJ
Kolb, M
Maher, TM
Nusser, R
Poletti, V
Richeldi, L
Vancheri, C
Wilsher, ML
Antoniou, KM
Behr, J
Bendstrup, E
Brown, KK
Corte, TJ
Cottin, V
Crestani, B
Flaherty, KR
Glaspole, IN
Grutters, J
Inoue, Y
Kondoh, Y
Kreuter, M
Johannson, KA
Ley, B
Martinez, FJ
Molina-Molina, M
Morais, A
Nunes, H
Raghu, G
Selman, M
Spagnolo, P
Taniguchi, H
Tomassetti, S
Valeyre, D
Wijsenbeek, M
Wuyts, WA
Wells, AU
Item Type: Journal Article
Abstract: RATIONALE: The level 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 sub-groups 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 el al. MEASUREMENTS AND MAIN RESULTS: 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 (70-89% likelihood), antifibrotic therapy was prescribed without requesting SLB. No significant mortality difference was observed between cases given a definite diagnosis of IPF (90-100% likelihood) and cases given a provisional high confidence diagnosis of IPF (HR 0.97, p=0.348, 95% CI 0.90-1.04). CONCLUSIONS: Most respiratory physicians prescribe antifibrotic therapy without requesting a 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.
Issue Date: 26-Jun-2019
Date of Acceptance: 25-Jun-2019
URI: http://hdl.handle.net/10044/1/71162
DOI: https://doi.org/10.1164/rccm.201903-0493OC
ISSN: 1073-449X
Publisher: American Thoracic Society
Journal / Book Title: American Journal of Respiratory and Critical Care Medicine
Copyright Statement: © 2019 by the American Thoracic Society.
Keywords: Antifibrotic therapy
Clinical practice guidelines
Idiopathic pulmonary fibrosis
Surgical lung biopsy
Working diagnosis
Antifibrotic therapy
Clinical practice guidelines
Idiopathic pulmonary fibrosis
Surgical lung biopsy
Working diagnosis
Respiratory System
11 Medical and Health Sciences
Publication Status: Published online
Conference Place: United States
Online Publication Date: 2019-06-26
Appears in Collections:National Heart and Lung Institute



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