Diagnosis and management of connective tissue disease-associated interstitial lung disease in Australia and New Zealand: A position statement from the Thoracic Society of Australia and New Zealand

doi:10.1111/resp.13977

. 2021 Jan;26(1):23-51.

doi: 10.1111/resp.13977. Epub 2020 Nov 24.

Diagnosis and management of connective tissue disease-associated interstitial lung disease in Australia and New Zealand: A position statement from the Thoracic Society of Australia and New Zealand

Adelle S Jee^{1

2

3}, Robert Sheehy^{4

5}, Peter Hopkins^{3

5

6}, Tamera J Corte^{1

2

3}, Christopher Grainge^{3

7}, Lauren K Troy^{1

2}, Karen Symons⁸, Lissa M Spencer⁹, Paul N Reynolds^{3

10

11}, Sally Chapman¹⁰, Sally de Boer¹², Taryn Reddy¹³, Anne E Holland^{3

14

15

16}, Daniel C Chambers^{3

5

6}, Ian N Glaspole^{3

8

17}, Helen E Jo^{1

2

3}, Jane F Bleasel^{2

18}, Jeremy P Wrobel^{19

20}, Leona Dowman^{3

14

21}, Matthew J S Parker^{2

3

18}, Margaret L Wilsher^{3

12

22}, Nicole S L Goh^{8

16

23

24}, Yuben Moodley^{3

25

26}, Gregory J Keir^{4

5}

Affiliations

¹ Department of Respiratory Medicine, Royal Prince Alfred Hospital, Sydney, NSW, Australia.
² Central Clinical School, University of Sydney, Sydney, NSW, Australia.
³ NHMRC Centre of Research Excellence in Pulmonary Fibrosis, Sydney, NSW, Australia.
⁴ Department of Respiratory Medicine, Princess Alexandra Hospital, Brisbane, QLD, Australia.
⁵ School of Medicine, University of Queensland, Brisbane, QLD, Australia.
⁶ Queensland Lung Transplant service, The Prince Charles Hospital, Brisbane, QLD, Australia.
⁷ Department of Respiratory Medicine, John Hunter Hospital, Newcastle, New South Wales, Australia.
⁸ Department of Respiratory Medicine, Alfred Hospital, Melbourne, VIC, Australia.
⁹ Department of Physiotherapy, Royal Prince Alfred Hospital, Sydney, NSW, Australia.
¹⁰ Department of Thoracic Medicine, Royal Adelaide Hospital, Adelaide, SA, Australia.
¹¹ Lung Research Laboratory, University of Adelaide, Adelaide, SA, Australia.
¹² Respiratory Services, Auckland District Health Board, Auckland, New Zealand.
¹³ Department of Medical Imaging, The Prince Charles Hospital, Brisbane, QLD, Australia.
¹⁴ Department of Allergy, Immunology and Respiratory Medicine, Monash University, Melbourne, VIC, Australia.
¹⁵ Department of Physiotherapy, Alfred Health, Melbourne, VIC, Australia.
¹⁶ Institute for Breathing and Sleep, Melbourne, VIC, Australia.
¹⁷ Central Clinical School, Monash University, Melbourne, VIC, Australia.
¹⁸ Department of Rheumatology, Royal Prince Alfred Hospital, Sydney, NSW, Australia.
¹⁹ Advanced Lung Disease Unit, Fiona Stanley Hospital, Perth, WA, Australia.
²⁰ Department of Medicine, University of Notre Dame Australia, Fremantle, WA, Australia.
²¹ Physiotherapy Department, Austin Health, Melbourne, VIC, Australia.
²² Faculty of Medicine and Health Sciences, University of Auckland, Auckland, New Zealand.
²³ Department of Respiratory Medicine, Austin Hospital, Melbourne, VIC, Australia.
²⁴ Melbourne Medical School, University of Melbourne, Melbourne, VIC, Australia.
²⁵ University of Western Australia, Institute for Respiratory Health, Perth, WA, Australia.
²⁶ Department of Respiratory Medicine, Fiona Stanley Hospital, Perth, WA, Australia.

PMID: 33233015
PMCID: PMC7894187
DOI: 10.1111/resp.13977

Diagnosis and management of connective tissue disease-associated interstitial lung disease in Australia and New Zealand: A position statement from the Thoracic Society of Australia and New Zealand

Adelle S Jee et al. Respirology. 2021 Jan.

. 2021 Jan;26(1):23-51.

doi: 10.1111/resp.13977. Epub 2020 Nov 24.

Authors

Affiliations

¹ Department of Respiratory Medicine, Royal Prince Alfred Hospital, Sydney, NSW, Australia.
² Central Clinical School, University of Sydney, Sydney, NSW, Australia.
³ NHMRC Centre of Research Excellence in Pulmonary Fibrosis, Sydney, NSW, Australia.
⁴ Department of Respiratory Medicine, Princess Alexandra Hospital, Brisbane, QLD, Australia.
⁵ School of Medicine, University of Queensland, Brisbane, QLD, Australia.
⁶ Queensland Lung Transplant service, The Prince Charles Hospital, Brisbane, QLD, Australia.
⁷ Department of Respiratory Medicine, John Hunter Hospital, Newcastle, New South Wales, Australia.
⁸ Department of Respiratory Medicine, Alfred Hospital, Melbourne, VIC, Australia.
⁹ Department of Physiotherapy, Royal Prince Alfred Hospital, Sydney, NSW, Australia.
¹⁰ Department of Thoracic Medicine, Royal Adelaide Hospital, Adelaide, SA, Australia.
¹¹ Lung Research Laboratory, University of Adelaide, Adelaide, SA, Australia.
¹² Respiratory Services, Auckland District Health Board, Auckland, New Zealand.
¹³ Department of Medical Imaging, The Prince Charles Hospital, Brisbane, QLD, Australia.
¹⁴ Department of Allergy, Immunology and Respiratory Medicine, Monash University, Melbourne, VIC, Australia.
¹⁵ Department of Physiotherapy, Alfred Health, Melbourne, VIC, Australia.
¹⁶ Institute for Breathing and Sleep, Melbourne, VIC, Australia.
¹⁷ Central Clinical School, Monash University, Melbourne, VIC, Australia.
¹⁸ Department of Rheumatology, Royal Prince Alfred Hospital, Sydney, NSW, Australia.
¹⁹ Advanced Lung Disease Unit, Fiona Stanley Hospital, Perth, WA, Australia.
²⁰ Department of Medicine, University of Notre Dame Australia, Fremantle, WA, Australia.
²¹ Physiotherapy Department, Austin Health, Melbourne, VIC, Australia.
²² Faculty of Medicine and Health Sciences, University of Auckland, Auckland, New Zealand.
²³ Department of Respiratory Medicine, Austin Hospital, Melbourne, VIC, Australia.
²⁴ Melbourne Medical School, University of Melbourne, Melbourne, VIC, Australia.
²⁵ University of Western Australia, Institute for Respiratory Health, Perth, WA, Australia.
²⁶ Department of Respiratory Medicine, Fiona Stanley Hospital, Perth, WA, Australia.

PMID: 33233015
PMCID: PMC7894187
DOI: 10.1111/resp.13977

Abstract

Pulmonary complications in CTD are common and can involve the interstitium, airways, pleura and pulmonary vasculature. ILD can occur in all CTD (CTD-ILD), and may vary from limited, non-progressive lung involvement, to fulminant, life-threatening disease. Given the potential for major adverse outcomes in CTD-ILD, accurate diagnosis, assessment and careful consideration of therapeutic intervention are a priority. Limited data are available to guide management decisions in CTD-ILD. Autoimmune-mediated pulmonary inflammation is considered a key pathobiological pathway in these disorders, and immunosuppressive therapy is generally regarded the cornerstone of treatment for severe and/or progressive CTD-ILD. However, the natural history of CTD-ILD in individual patients can be difficult to predict, and deciding who to treat, when and with what agent can be challenging. Establishing realistic therapeutic goals from both the patient and clinician perspective requires considerable expertise. The document aims to provide a framework for clinicians to aid in the assessment and management of ILD in the major CTD. A suggested approach to diagnosis and monitoring of CTD-ILD and, where available, evidence-based, disease-specific approaches to treatment have been provided.

Keywords: clinical diagnosis and management; collagen vascular disease; connective tissue disease; interstitial lung disease.

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Figures

**Figure 1**
Common radiological patterns of interstitial lung disease (ILD) in connective tissue disease (CTD). (A) Non‐specific interstitial pneumonia (NSIP): ground‐glass change and limited reticulation; predominantly basal, subpleural distribution. (B) Usual interstitial pneumonia (UIP): subpleural, basal predominant reticulation with honeycomb change. No ground‐glass change, nodules or air‐trapping (features ‘inconsistent’ with radiological UIP). (C) Organizing pneumonia (OP): dense, patchy predominantly peribronchial consolidation affecting the lower lobes bilaterally and symmetrically. (D) Acute interstitial pneumonia (AIP): widespread ground‐glass infiltrates in a patient with previously undiagnosed systemic lupus erythematosus (SLE) presenting with acute respiratory failure. (E) Lymphocytic interstitial pneumonia (LIP): variably sized thin‐walled cysts. Ground‐glass change and centrilobular nodules may also occur (not seen here).

**Figure 2**
Suggested algorithm for the diagnosis and management of connective tissue disease‐associated interstitial lung disease (CTD‐ILD). ^†For detailed discussion, see the section ‘Deciding when to treat’.

**Figure 3**
(A) A simple staging system for systemic sclerosis‐associated interstitial lung disease (SSc‐ILD). (B) Survival curves for SSc‐ILD patients with ‘limited’ versus ‘extensive’ disease (hazard ratio (HR) = 3.46; P < 0.0005) (Adapted from Goh *et al*., ⁵⁴ with permission).

See this image and copyright information in PMC

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References

1. Wells A, Denton CP. Pulmonary complications of connective tissue disease. Semin. Respir. Crit. Care Med. 2019; 40: 145–6. - PubMed
1. Raghu G, Remy‐Jardin M, Myers JL, Richeldi L, Ryerson CJ, Lederer DJ, Behr J, Cottin V, Danoff SK, Morell F et al Diagnosis of idiopathic pulmonary fibrosis. An official ATS/ERS/JRS/ALAT clinical practice guideline. Am. J. Respir. Crit. Care Med. 2018; 198: e44–68. - PubMed
1. Jo HE, Troy LK, Keir G, Chambers DC, Holland A, Goh N, Wilsher M, de Boer S, Moodley Y, Grainge C et al Treatment of idiopathic pulmonary fibrosis in Australia and New Zealand: a position statement from the Thoracic Society of Australia and New Zealand and the Lung Foundation Australia. Respirology 2017; 22: 1436–58. - PubMed
1. Suzuki A, Kondoh Y, Fischer A. Recent advances in connective tissue disease related interstitial lung disease. Expert Rev. Respir. Med. 2017; 11: 591–603. - PubMed
1. Mira‐Avendano I, Abril A, Burger CD, Dellaripa PF, Fischer A, Gotway MB, Lee AS, Lee JS, Matteson EL, Yi ES et al Interstitial lung disease and other pulmonary manifestations in connective tissue diseases. Mayo Clin. Proc. 2019; 94: 309–25. - PubMed

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[1] Wells A, Denton CP. Pulmonary complications of connective tissue disease. Semin. Respir. Crit. Care Med. 2019; 40: 145–6. - PubMed

[2] Wells A, Denton CP. Pulmonary complications of connective tissue disease. Semin. Respir. Crit. Care Med. 2019; 40: 145–6. - PubMed

[3] Raghu G, Remy‐Jardin M, Myers JL, Richeldi L, Ryerson CJ, Lederer DJ, Behr J, Cottin V, Danoff SK, Morell F et al Diagnosis of idiopathic pulmonary fibrosis. An official ATS/ERS/JRS/ALAT clinical practice guideline. Am. J. Respir. Crit. Care Med. 2018; 198: e44–68. - PubMed

[4] Raghu G, Remy‐Jardin M, Myers JL, Richeldi L, Ryerson CJ, Lederer DJ, Behr J, Cottin V, Danoff SK, Morell F et al Diagnosis of idiopathic pulmonary fibrosis. An official ATS/ERS/JRS/ALAT clinical practice guideline. Am. J. Respir. Crit. Care Med. 2018; 198: e44–68. - PubMed

[5] Jo HE, Troy LK, Keir G, Chambers DC, Holland A, Goh N, Wilsher M, de Boer S, Moodley Y, Grainge C et al Treatment of idiopathic pulmonary fibrosis in Australia and New Zealand: a position statement from the Thoracic Society of Australia and New Zealand and the Lung Foundation Australia. Respirology 2017; 22: 1436–58. - PubMed

[6] Jo HE, Troy LK, Keir G, Chambers DC, Holland A, Goh N, Wilsher M, de Boer S, Moodley Y, Grainge C et al Treatment of idiopathic pulmonary fibrosis in Australia and New Zealand: a position statement from the Thoracic Society of Australia and New Zealand and the Lung Foundation Australia. Respirology 2017; 22: 1436–58. - PubMed

[7] Suzuki A, Kondoh Y, Fischer A. Recent advances in connective tissue disease related interstitial lung disease. Expert Rev. Respir. Med. 2017; 11: 591–603. - PubMed

[8] Suzuki A, Kondoh Y, Fischer A. Recent advances in connective tissue disease related interstitial lung disease. Expert Rev. Respir. Med. 2017; 11: 591–603. - PubMed

[9] Mira‐Avendano I, Abril A, Burger CD, Dellaripa PF, Fischer A, Gotway MB, Lee AS, Lee JS, Matteson EL, Yi ES et al Interstitial lung disease and other pulmonary manifestations in connective tissue diseases. Mayo Clin. Proc. 2019; 94: 309–25. - PubMed

[10] Mira‐Avendano I, Abril A, Burger CD, Dellaripa PF, Fischer A, Gotway MB, Lee AS, Lee JS, Matteson EL, Yi ES et al Interstitial lung disease and other pulmonary manifestations in connective tissue diseases. Mayo Clin. Proc. 2019; 94: 309–25. - PubMed

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Diagnosis and management of connective tissue disease-associated interstitial lung disease in Australia and New Zealand: A position statement from the Thoracic Society of Australia and New Zealand

Affiliations

Diagnosis and management of connective tissue disease-associated interstitial lung disease in Australia and New Zealand: A position statement from the Thoracic Society of Australia and New Zealand

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