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

Jo, HE, Glaspole, I, Goh, N, Hopkins, PMA, Moodley, Y, Reynolds, PN, Chapman, S, Walters, EH ORCID: 0000-0002-0993-4374, Zappala, C, Allan, H, Macansh, S, Grainge, C, Keir, GJ, Hayen, A, Henderson, D, Klebe, S, Heinze, SB, Miller, A, Rouse, HC, Duhig, E, Cooper, WA, Mahar, AM, Ellis, S, McCormack, SR, Ng, B, Godbolt, DB and Corte, TJ 2018 , 'Implications of the diagnostic criteria of idiopathic pulmonary fibrosis in clinical practice: analysis from the Australian Idiopathic Pulmonary Fibrosis Registry' , Respirology, vol. 24, no. 4 , pp. 361-368 , doi:

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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 Type: Article
Authors/Creators: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
Keywords: honeycombing, idiopathic pulmonary fibrosis, multidisciplinary, registry
Journal or Publication Title: Respirology
Publisher: Blackwell Science
ISSN: 1323-7799
DOI / ID Number:
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Copyright 2018 Asian Pacific Society of Respirology

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