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Implications of the diagnostic criteria of idiopathic pulmonary fibrosis in clinical practice: analysis from the Australian Idiopathic Pulmonary Fibrosis Registry

Citation

Jo, HE and Glaspole, I and Goh, N and Hopkins, PMA and Moodley, Y and Reynolds, PN and Chapman, S and Walters, EH and Zappala, C and Allan, H and Macansh, S and Grainge, C and Keir, GJ and Hayen, A and Henderson, D and Klebe, S and Heinze, SB and Miller, A and Rouse, HC and Duhig, E and Cooper, WA and Mahar, AM and Ellis, S and McCormack, SR and Ng, B and Godbolt, DB and Corte, TJ, Implications of the diagnostic criteria of idiopathic pulmonary fibrosis in clinical practice: analysis from the Australian Idiopathic Pulmonary Fibrosis Registry, Respirology, 24, (4) pp. 361-368. ISSN 1323-7799 (2018) [Refereed Article]

Copyright Statement

Copyright 2018 Asian Pacific Society of Respirology

DOI: doi:10.1111/resp.13427

Abstract

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.

Item Details

Item Type:Refereed Article
Keywords:honeycombing, idiopathic pulmonary fibrosis, multidisciplinary, registry
Research Division:Medical and Health Sciences
Research Group:Cardiorespiratory Medicine and Haematology
Research Field:Respiratory Diseases
Objective Division:Health
Objective Group:Clinical Health (Organs, Diseases and Abnormal Conditions)
Objective Field:Respiratory System and Diseases (incl. Asthma)
UTAS Author:Walters, EH (Professor Haydn Walters)
ID Code:130932
Year Published:2018
Web of Science® Times Cited:3
Deposited By:Medicine
Deposited On:2019-02-20
Last Modified:2019-04-26
Downloads:0

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