Implications of the diagnostic criteria of idiopathic pulmonary fibrosis in clinical practice: Analysis from the Australian Idiopathic Pulmonary Fibrosis Registry
Open accessAbstract
Background and objective: Current guidelines for the diagnosis of idiopathic pulmonary fibrosis (IPF) provide specific criteria for diagnosis in the setting of multidisciplinary discussion (MDD). We evaluate the utility and reproducibility of these diagnostic guidelines, using clinical data from the Australian IPF Registry. Methods: All patients enrolled in the registry undergo a diagnostic review whereby international IPF guidelines are applied via a registry MDD. We investigated the clinical applicability of these guidelines with regard to: (i) adherence to guidelines, (ii) Natural history of IPF diagnostic categories and (iii) Concordance for diagnostic features. Results: A total of 417 participants (69% male, 70.6 ± 8.0 years) with a clinical diagnosis of IPF underwent MDD. The 23% of participants who did not meet IPF diagnostic criteria displayed identical disease behaviour to those with confirmed IPF. Honeycombing on radiology was associated with a worse prognosis and this translated into poorer prognosis in the ‘definite’ IPF group. While there was moderate agreement for IPF diagnostic categories, agreement for specific radiological features, other than honeycombing, was poor. Conclusion: In clinical practice, physicians do not always follow IPF diagnostic guidelines. We demonstrate a cohort of IPF patients who do not meet IPF diagnostic guideline criteria, based largely on their radiology and lack of lung biopsy, but who have outcomes identical to those with IPF.
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Self-citation
- Authors
- Jo, H. E., Glaspole, I., Goh, N., Hopkins, P. M. A., Moodley, Y., Reynolds, P. N., Chapman, S., Walters, E. H., Zappala, C., Allan, H., Macansh, S., Grainge, C., Keir, G. J., Hayen, A., Henderson, D., Klebe, S., Heinze, S. B., Miller, A., Rouse, H. C., Duhig, E., Cooper, W. A., Mahar, A. M., Ellis, S., McCormack, S. R., Ng, B., Godbolt, D. B., & Corte, T. J.
- Affiliations
- The University of Sydney (AU)
- Austin Hospital (AU)
- Royal Adelaide Hospital (AU)
- Royal Brisbane and Women's Hospital (AU)
- Lung Foundation Australia (AU)
- Princess Alexandra Hospital (AU)
- University of Technology Sydney (AU)
- Flinders Medical Centre (AU)
- The Royal Melbourne Hospital (AU)
- Royal North Shore Hospital (AU)
- St Vincent's Hospital Melbourne (AU)
- Sullivan Nicolaides Pathology (AU)
- Royal Prince Alfred Hospital (AU)
- The Alfred Hospital (AU)
- Prince Charles Hospital (AU)
- Corresponding author
- Jo, Helen E.
- ORCID
- Jo, Helen E.
- Glaspole, Ian
- Goh, Nicole
- Hopkins, Peter M.A.
- Moodley, Yuben
- Reynolds, Paul N.
- Chapman, Sally
- Walters, Eugene Haydn
- Zappala, Christopher
- Allan, Heather
- Grainge, Christopher
- Keir, Gregory J.
- Hayen, Andrew
- Henderson, Douglas
- Klebe, Sonja
- Heinze, Stefan B.
- Duhig, Edwina
- Cooper, Wendy A.
- Mahar, Annabelle M.
- Ellis, Samantha
- Ng, Bernard
- Godbolt, David B.
- Corte, Tamera J.
- Venue
- Respirology
- Publisher
- Wiley
- Published
- 2018-10-17
- Volume
- 24
- Issue
- 4
- Pages
- 361-368
- ISSN
- 1323-7799 (ISSN-L)
- 1440-1843
- OpenAlex
- W2895937407
- Cited by
- 28
- References
- 29
- Citations by year
- 2026: 1 · 2025: 1 · 2024: 3 · 2023: 1 · 2022: 3 · 2021: 4 · 2020: 10 · 2019: 4 · 2018: 1
- FWCI
- 3.58
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Respirology (publishedVersion)
- PubMed (publishedVersion)
- eCite Digital Repository (University of Tasmania) (submittedVersion)
- UTAS Research Repository (submittedVersion)
- Adelaide Research & Scholarship (AR&S) (University of Adelaide) (submittedVersion)
- Figshare (submittedVersion, cc-by)
- Minerva Access (University of Melbourne) (submittedVersion)
- UTS ePRESS (University of Technology Sydney) (submittedVersion)
- NOVA (University of Newcastle Australia) (submittedVersion)
- Funders
- National Health and Medical Research Council (APP1066128)
- Topics
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Occupational and environmental lung diseases
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Medical Imaging and Pathology Studies
- Keywords
- idiopathic pulmonary fibrosis, diagnostic criteria, multidisciplinary discussion, guideline adherence, diagnostic agreement, honeycombing, radiological features, lung biopsy, natural history (disease), prognosis
- MeSH terms
- Aged, Australia, Biopsy, Female, Humans, Lung, Male, Middle Aged, Prognosis, Registries, Radiography, Thoracic, Reproducibility of Results, Cohort Studies, Practice Guidelines as Topic, Guideline Adherence, Idiopathic Pulmonary Fibrosis
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