Diagnostic accuracy of a clinical diagnosis of idiopathic pulmonary fibrosis: an international case–cohort study

Abstract

We conducted an international study of idiopathic pulmonary fibrosis (IPF) diagnosis among a large group of physicians and compared their diagnostic performance to a panel of IPF experts.A total of 1141 respiratory physicians and 34 IPF experts participated. Participants evaluated 60 cases of interstitial lung disease (ILD) without interdisciplinary consultation. Diagnostic agreement was measured using the weighted kappa coefficient (κw). Prognostic discrimination between IPF and other ILDs was used to validate diagnostic accuracy for first-choice diagnoses of IPF and were compared using the C-index.A total of 404 physicians completed the study. Agreement for IPF diagnosis was higher among expert physicians (κw=0.65, IQR 0.53–0.72, p<0.0001) than academic physicians (κw=0.56, IQR 0.45–0.65, p<0.0001) or physicians with access to multidisciplinary team (MDT) meetings (κw=0.54, IQR 0.45–0.64, p<0.0001). The prognostic accuracy of academic physicians with >20 years of experience (C-index=0.72, IQR 0.0–0.73, p=0.229) and non-university hospital physicians with more than 20 years of experience, attending weekly MDT meetings (C-index=0.72, IQR 0.70–0.72, p=0.052), did not differ significantly (p=0.229 and p=0.052 respectively) from the expert panel (C-index=0.74 IQR 0.72–0.75).Experienced respiratory physicians at university-based institutions diagnose IPF with similar prognostic accuracy to IPF experts. Regular MDT meeting attendance improves the prognostic accuracy of experienced non-university practitioners to levels achieved by IPF experts.

Managing disease behaviour: A team approach

“…Multidisciplinary decision‐making is now routine practice in ILD but perhaps it is time to include the patient's voice in the form of validated symptom and quality of life measures, not just in clinical trials but in every day clinical practice. The resultant consensus decisions may be more meaningful for those who benefit from or bear the impact of treatment.…”

Idiopathic pulmonary fibrosis: airway volume measurement identifies progressive disease on computed tomography scans

“…Whilst antifibrotic therapy can slow disease progression [1,2], the ability to determine an individual's disease course is limited to measurement of pulmonary function ( pulmonary function testing (PFT)) and visual assessment of computed tomography (CT) scans, both of which have limitations. PFT is affected by patient technique and operator experience [3]; CT scans rely on radiologist expertise to correctly interpret disease stability/progression and are subject to interobserver variability [4]. Recently, automated analysis of blood vessel volume has been shown to predict disease progression [5].…”

“…Specific airway volume (siV aw ) was calculated by dividing the airway volume by lung volume. This allowed for interpatient comparison of airway volume in patients with different lung volumes and intrapatient comparison, given that lung volumes were expected to decline between imaging [4]. The study was approved by the ethics committee at our institution (ref.…”

Interstitielle Lungenerkrankungen: epidemiologische Herausforderungen, Register und Biobanken

“…Dazu kommt die individuelle Interpretation der klinischen und radiologischen Befunde als beschränkender Faktor der Diagnose: In einer internationalen Studie, bei der die diagnostische Genauigkeit von etwa. 1 100 Pneumologen und 34 IPF-Experten im Zusammenhang mit IPF und ILD untersucht wurde, gab es wesentliche Unterschiede zwischen den oben genannten Medizinergruppen bei der Diagnose der ILDs [11]. Aufgrund solcher diagnostischen Schwierigkeiten wurde vor Kurzem ein White Paper der Fleischner Society zur IPF-Diagnose veröffentlicht: Die wichtigste Erkenntnis dieses Artikels ist, dass Patienten mit typischen klinischen Charakteristika für eine IPF und einem wahrscheinlichen (probablen) UIP-Muster (d.h. das Fehlen der typischen Honigwaben) in HRCT als IPF-Patienten eingestuft werden können [12].…”