Diagnosis and management of idiopathic pulmonary fibrosis: Thoracic Society of Australia and New Zealand and Lung Foundation Australia position statements summary

Jo*, Helen E; Prasad*, Jyotika D; Troy, Lauren K; Mahar, Annabelle; Bleasel, Jane; Ellis, Samantha J; Chambers, Daniel C; Holland, Anne E; Lake, Fiona R; Keir, Gregory; Goh, Nicole S; Wilsher, Margaret; de Boer, Sally; Moodley, Yuben; Grainge, Christopher; Whitford, Helen M; Chapman, Sally A; Reynolds, Paul N; Beatson**, David; Jones, Leonie J; Hopkins, Peter; Allan, Heather M; Glaspole***, Ian; Corte***, Tamera J

doi:10.5694/mja17.00799

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Abstract

Introduction: Idiopathic pulmonary fibrosis (IPF) is a fibrosing interstitial lung disease associated with debilitating symptoms of dyspnoea and cough, resulting in respiratory failure, impaired quality of life and ultimately death. Diagnosing IPF can be challenging, as it often shares many features with other interstitial lung diseases. In this article, we summarise recent joint position statements on the diagnosis and management of IPF from the Thoracic Society of Australia and New Zealand and Lung Foundation Australia, specifically tailored for physicians across Australia and New Zealand.

Main suggestions:

A comprehensive multidisciplinary team meeting is suggested to establish a prompt and precise IPF diagnosis.
Antifibrotic therapies should be considered to slow disease progression. However, enthusiasm should be tempered by the lack of evidence in many IPF subgroups, particularly the broader disease severity spectrum.
Non-pharmacological interventions including pulmonary rehabilitation, supplemental oxygen, appropriate treatment of comorbidities and disease-related symptoms remain crucial to optimal management.
Despite recent advances, IPF remains a fatal disease and suitable patients should be referred for lung transplantation assessment.

Helen E Jo*¹
Jyotika D Prasad*²^,³
Lauren K Troy¹
Annabelle Mahar¹
Jane Bleasel⁴
Samantha J Ellis²
Daniel C Chambers⁵^,⁶
Anne E Holland²^,⁷
Fiona R Lake⁸
Gregory Keir⁹
Nicole S Goh¹⁰
Margaret Wilsher¹¹^,¹²
Sally de Boer¹³
Yuben Moodley⁸
Christopher Grainge¹⁴
Helen M Whitford²
Sally A Chapman¹⁵
Paul N Reynolds¹⁵
David Beatson**¹⁶
Leonie J Jones¹⁴
Peter Hopkins¹⁷
Heather M Allan¹⁸
Ian Glaspole***²^,¹⁹
Tamera J Corte***¹

1 Royal Prince Alfred Hospital, Sydney, NSW
2 Alfred Hospital, Melbourne, VIC
3 Royal Melbourne Hospital, Melbourne, VIC
4 University of Sydney, Sydney, NSW
5 University of Queensland, Brisbane, QLD
6 Prince Charles Hospital, Brisbane, QLD
7 La Trobe University, Melbourne, VIC
8 University of Western Australia, Perth, WA
9 Princess Alexandra Hospital, Brisbane, QLD
10 Austin Health, Melbourne, VIC
11 Auckland District Health Board, Auckland, NZ
12 University of Auckland, Auckland, NZ
13 Auckland City Hospital, Auckland, NZ
14 John Hunter Hospital, Newcastle, NSW
15 Royal Adelaide Hospital, Adelaide, SA
16 Auckland, NZ
17 Queensland Lung Transplant Service, Prince Charles Hospital, Brisbane, QLD
18 Lung Foundation Australia, Brisbane, QLD
19 Monash University, Melbourne, VIC

Correspondence: helen.jo@sydney.edu.au

* Joint first authors

** Deceased

*** Joint senior authors

Acknowledgements:

David Beatson was a well known New Zealand journalist, broadcaster and editor. Among his many roles, he served as Prime Minister’s chief press officer, and chair of NZ on Air. A thoughtful, erudite and engaging patient, David died from IPF on 21 September 2017. We acknowledge his important contribution as patient author on this article.

Competing interests:

For competing interest declarations, see online .

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Diagnosis and management of idiopathic pulmonary fibrosis: Thoracic Society of Australia and New Zealand and Lung Foundation Australia position statements summary

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