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Australasian Interstitial Lung Disease Registry (AILDR)
A prospective clinical Registry for Interstitial Lung Disease (ILD)
AILDR recruits patients with all ILD subtypes, reflecting real world practice at clinics across Australia and New Zealand with dual objectives:
- To provide a valuable resource for high quality ILD research
- To improve care for ILD patients across Australia and NZ
The AILDR was established in response to growing calls for a national clinical Registry to better understand Interstitial Lung Disease patterns, standardise care and provide relevant longitudinal data. Commencing with four pilot sites in 2016, there are now 21 sites participating across Australia and New Zealand with more than 2700 participants recruited and followed-up regularly every 6 months.
Inclusion criteria for participants includes age 18 years of age, ability to provide written informed consent, and diagnosis of ILD according to American Thoracic Society/European Respiratory Society (ATS/ERS) criteria. The core data recorded on the registry includes:
- Basic demographic data
- Clinical data
- Medications
- Oxygen use
- Pulmonary function tests
- Other Investigations
More details for researchers planning to use the AILDR can be obtained by contacting the AILDR Project Manager.
Data linkage to the following AIHW datasets and state-based linkage units is planned to facilitate future studies of health service use, epidemiology, and needs analysis:
- Medicare and PBS
- National Death Index
- National Coronial Information System
- The Centre for Health Record Linkage (CHeReL)
- The Centre for Victorian Data Linkage (CVDL)
- Data Linkage Queensland (DLQ)
- WA Data Linkage System (WADLS)
- SA-NT DataLink
The Registry is linked to biobanks at registry sites collecting serum, bronchoalveolar lavage fluid and lung tissue according to an agreed Biobanking SOP (PDF, 183KB).
One of the main aims of the Registry is to provide a valuable resource for researchers and to enable collaborative research. It is advisable for researchers considering using the data to speak to one of the chairs early in the process so that we can assist with applications advice. Publications should adhere to the publication and authorship policy.
For access to single site data:
- Principal Investigators assume responsibility for own institution’s approval.
- Access does not require Steering Committee approval.
- Acknowledgement of AILDR is encouraged for presentations.
- Publications should adhere to the publication and authorship policy (PDF, 120.3KB).
To access multi-site data there are two steps to obtaining Steering Committee (SC) approval:
Step 1 - Submit the following documents by email to the AILDR Project Manager to obtain “In-Principle” approval from the AILDR Steering Committee and reserve the study:
- a cover letter (addressed to The Chair, Australasian ILD Registry)
- study proposal (no more than 2 pages)
- list of data fields required (please refer to AILDR data dictionary (PDF, 441.1KB).
Research proposals will be reviewed by the AILDR Steering Committee at the next scheduled meeting to ensure that the proposal is unique and addresses a specific and sound scientific question. The Executive of the Steering Committee will subsequently vote to approve the study proposal. Researchers will be informed of the outcome to the application within 4 weeks of the meeting.
Step 2 - Submit the following documents by email to the AILDR Project Manager to obtain final Steering Committee approval:
- HREC approval letter
- Study protocol
- Budget (if applicable)
The Steering Committee will consider the full application and subject to queries being addressed. Researchers will be notified of final approval as soon as possible, usually within 4-6 weeks from receipt of the study protocol.
Our Research
Publications:
Moore, I., Wrobel, J., Rhodes, J. et al. Australasian interstitial lung disease registry (AILDR): objectives, design and rationale of a binational prospective database. BMC Pulm Med 20, 257 (2020). (Download PDF)
Current studies
Current studies:
- Gender bias project in collaboration with CARE PF Registry - Dr Robert Sheehy
- Clinical Validation of a Hypersensitivity Pneumonitis (HP) Exposure Questionnaire - Dr Hayley Barnes
- CT Deep Learning Algorithm to predict prognosis in ILD - Dr John Mackintosh, A/Prof Tamera Corte
- Interstitial Pulmonary Fibrosis descriptive analysis - Prof Frank Thien
- Implementation of TSANZ/LFA Position Statement of ILD Diagnosis in Clinical Practice – A/Prof Yet Khor
- Disease Behaviour of Unclassifiable ILD – A/Prof Yet Khor
- Myositis-related ILD – an Australasian perspective – Dr Matthew Parker
- The use of nintedanib in a cohort of Australasian patients with non-Idiopathic Pulmonary Fibrosis Interstitial Lung Disease – Dr Matthew Parker
Abstracts/Presentations
- Australia and New Zealand interstitial lung disease registry (ANZ ILD) 2021 update - Progress during the pandemic. Moore I. et al. TSANZSRS ASM 2022 and ATS 2022.
- Idiopathic pulmonary fibrosis-eastern health Victoria benchmarked against national population. Chee M et al. et al. TSANZSRS ASM 2022.
- Registry for better understanding of ILD (RE-BUILD) Mobile application. Glenn LM. et al. TSANZSRS ASM 2022 and ATS 2022.
- Significant deterioration in IPF and hypersensitivity-pneumonitis patients from Western Australia. Wigston C. et al. TSANZSRS ASM 2022.
- Real World Interstitial Lung Disease Experience - Data from the Australasian Interstitial Lung Disease Registry (AILDR) – Moore, I. TSANZSRS ASM 2020 and ATS 2020.
Registry for Better Understanding of ILD: RE-BUILD Smartphone App
AILDR Investigators have developed a digital mobile health solution for patients with ILD which has the potential to transform the way participants self-manage their health and interact with researchers particularly for patients living rurally or remotely who are not close to ILD centres.
The RE-BUILD app prototype commenced real user testing in November 2021 at three ILD research centres in NSW and Victoria (Royal Prince Alfred Hospital, Austin Health, and The Alfred Hospital).
Participants in the app’s pilot study will help Investigators test the app’s functions by sharing their health information, spirometry results, and submitting questionnaires over a 6-month period. The app will make use of smartphones in-built technology to gather invaluable physical activity data and air quality at participants’ location while they go about their daily lives. The app utilises smart digital nudges - offering suggestions, reminders and positive reinforcement that are specifically designed to help users monitor and improve their health status. Other novel features include a straightforward feed to high-quality informative resources from trusted sources and the ability for to users stay up to date with clinical trials and research studies all without leaving the app.
Investigators hope that following this pilot study the RE-BUILD app will be available for adoption across all Australasian ILD Registry sites in Australia and New Zealand.
The Registry was designed to build upon the Lung Foundation Australia’s success with development and administration of the Australian Idiopathic Pulmonary Fibrosis Registry.
The University of Sydney manages operations including IT, financial and legal aspects. Lung Foundation Australia provide governance and maintain the confidence of all parties. The AILDR Steering Committee provide oversight, direction, and guidance to ensure sound operations, to promote effective use of the data and to facilitate collaboration across institutions, States, disciplines and internationally. Membership of the Committee is broadly multi-disciplinary and includes representatives from all participating Australian states and New Zealand. Advisors and consumers are called upon to advise the Committee as required.
The AILDR is supported by the Centre of Research Excellence in Pulmonary Fibrosis (funded by the NHMRC, Lung Foundation Australia, Three Lakes Foundation, anonymous philanthropy, and Foundation partner Boehringer Ingelheim) and participating centres.
There are 21 sites participating across Australia and New Zealand.
Australian ILD Registry Centres |
|
State |
Site |
NSW |
Concord Hospital |
John Hunter Hospital |
|
Royal Prince Alfred Hospital |
|
Sutherland Hospital |
|
Westmead Hospital |
|
QLD |
Princess Alexandra Hospital |
The Prince Charles Hospital |
|
SA |
Flinders Medical Centre |
Royal Adelaide Hospital |
|
The Queen Elizabeth Hospital |
|
VIC |
The Alfred Hospital |
The Austin Hospital |
|
Eastern Health |
|
Royal Melbourne Hospital |
|
St Vincent’s Melbourne Hospital |
|
Western Health |
|
WA |
Fiona Stanley Hospital |
Sir Charles Gairdner Hospital |
|
New Zealand ILD Registry Centres |
|
City |
Site |
Auckland |
Auckland City Hospital |
Christchurch |
Christchurch Hospital |
Hamilton |
Waikato Hospital |
Clinicians that are interested in participating in the Australasian Interstitial Lung Disease Registry (AILDR) should complete and submit the AILDR New Site Application Form.
Our people
Co-Chairs:
AILDR Steering Committee
Executive:
- Prof Dan Chambers
- Dr Sally de Boer
- A/Prof Nicole Goh
- A/Prof Chris Grainge
- Dr Greg Keir
- A/Prof Yet Khor
- A/Prof Yuben Moodley
- Prof Paul Reynolds
- Michelle Galbraith CNS
- A/Prof Lauren Troy
- Prof Margaret Wilsher
- Dr Jeremy Wrobel
Regular Members:
- Dr Hayley Barnes
- Dr Laura Glenn
- Dr Adelle Jee
- Dr John Mackintosh
- Dr Irene Moore
- Sacha Macansh
- Dr Robert Sheehy
- Dr Alan Teoh
- Prof Frank Thien
Advisory Members:
- Epidemiology: Dr Vidya Navaratnam
- Palliative Care: A/Prof Natasha Smallwood
- Pathology: Prof Wendy Cooper
- Radiology: A/Prof Samantha Ellis, Dr David Milne
- Translation and Patient Engagement: Prof Anne Holland
Australian IPF Registry
The AIPFR has now been finalised. Data collected from 867 generous participants living with IPF, includes patient reported outcome data, pulmonary function data, HRCT scans and blood samples, continues to be available for research. A foundation platform of the Centre of Research Excellence in Pulmonary Fibrosis (CRE-PF), the AIPFR has been a collaborative project since inception. The success of this collaborative model is illustrated in AIPFR data facilitating diverse research projects resulting in more than 24 papers published in peer reviewed journals.
Participant recruitment started in February 2011 and ended December 2020. Data collection finished 31 December 2021, with vitality and transplant status continuing to be updated where possible. All Stages of the AIPFR cohort are longitudinal with data collection until death or transplant. Where participants indicated that they were no longer able to complete questionnaires, physician and test results continued to be collected. HRCT scans and blood samples were also routinely collected from AIPFR participants. See “Data Collection” section below for further detail.
The AIPFR stages and numbers of participants recruited are summarised in Table 1. For information on the AIPFR Stages and data, scan and sample collection intervals please refer to the AIPFR protocol v1 12 May 2017.
Table 1: AIPFR recruitment summary.
AIPFR |
Number Participants Recruited |
Blood substudy (# participants) |
AIPFR Stage description of recruitment** |
|---|---|---|---|
Stage 1 |
679 |
202 |
Australia wide, clinical diagnosis any stage of disease |
Stage 2 |
167 |
167* |
ILD Specialist centre, recent MDT diagnosis of IPF |
Stage 3 |
21 |
N/A |
ILD Specialist centre, any other patient, IPF diagnosis |
Total |
867 |
369 |
|
Non screening log |
104 |
N/A |
Patient invited to join AIPFR who declined, any stage, |
*106 participants with at least 4 serial blood samples
** Refer to the AIPFR Protocol for further information (see link above)
AIPFR Data Collection
Table 2: Australian IPF Registry data collection Stages 1-3
| Data source | Frequency | Summary of data content collected |
|---|---|---|
i. Participant questionnaire Stages 1-3 |
Current information collection Repeated every 6-months (At 0, 3, 6-months, then 6 monthly for Stage 2-3) |
Current information on:
|
ii. Physician questionnaire Stage 1 ONLY |
Repeated every 6-months but collecting distinct information for every patient visit during this time (At 0, 3, 6-months, then 6 monthly for Stage 2-3) |
|
iii. Clinical investigations Stage 1 listed investigations collected Stage 2-3 ONLY specified investigations collected |
Ongoing although new investigations are identified every 6-months
(At 0, 3, 6-months, then 6 monthly for Stage 2-3) |
|
iv. Expert panel review Stage 1 ONLY |
When a HRCT scan and/or surgical biopsy are identified for a participant |
|
v. Treatment questionnaire Stage 2-3 ONLY |
At 0, 3, 6-months, then 6 monthly |
Current information on:
|
vi. Treatment physician form Stage 2-3 ONLY |
At 0, 3, 6-months, then 6 monthly |
Current information on:
|
vii. Vitality status Stage 1, 2 and 3 |
At advice/collection | Vitality status fields including any information on Date of death and/or transplant |
AIPFR HRCT scans
AIPFR High Resolution Computed Tomography (HRCT) scans are available to approved researchers in a deidentified format via the central XNAT imaging platform. A total of 809 HRCT scans are available for AIPFR participants. Most AIPFR participants have one HRCT collected, however on occasion multiple scans are available for a single participant. In Stage 2, the HRCT reviewed by the MDT for diagnosis was collected. Scans cannot be copied or downloaded from XNAT. For further information see AILDR Radiology Repository section.
AIPFR Linked Biobank
A total of 396 AIPFR participants have at least one blood sample collected. Plasma, serum and RNA samples were collected at the first sample collection. For Stage 2 participants plasma and serum were collected at subsequent collections. The number and frequency of sample collection is detailed in the AIPFR protocol v1 12 May 2017.
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Acknowledgements
AIPFR was originally established through the generous support of a philanthropic family and the Royal Hobart Hospital Research Foundation.
The Australian IPF Registry was supported by long term Foundation partners Boehringer Ingelheim and Roche Products Pty Ltd. Financial support for the Australian IPF Registry was also proudly provided in the form of an unrestricted educational grants.
AIPFR also gratefully acknowledges the generous contribution of the members of the Registry of Steering Committee (now PIC), Radiology and Histopathology Review panel members together with AIPFR Coordinators, Data Mangers, and Manager.
One of the main aims of the Australian IPF Registry (AIPFR) is to provide a valuable resource for researchers and to facilitate collaborative research. It is advisable for researchers considering using AIPFR data to speak to one of the AIPFR Co-chairs early in the process so that they can provide advice on applications. Researchers approved to use AIPFR data may also be asked to include and work with an AIPFR Principal Investigator to advise on the appropriate use and interpretation of AIPFR data.
There are two steps to obtain AIPFR Principal Investigator approval and access AIPFR data, HRCT scans and/or biobank samples:
- Step 1
Submit the following documents by email to the AILDR Project Manager to obtain “In-Principle” approval from the AIPFR Principal Investigator Committee (PIC) and reserve the study:
- Cover letter addressed to the AIPFR PIC Co-Chair, Australian IPF Registry
- Summary of study proposal, approximately 2 pages
- List of data areas requested - please refer to Table 2: Australian IPF Registry data collection Stages 1-3
- Proposed authorship
- Funding or explanation of how resources will be made available to undertake the research project.
The AIPFR PIC will review and consider research proposals to ensure that the proposal is unique and addresses a specific and sound scientific question. The AIPFR PIC will review proposals as they are received.
- Step 2
Submit the following documents by email to the AILDR Project Manager to obtain final AIPFR PIC approval:
- Study protocol
- Approval letter from HREC local to the institution/s where the research will be undertaken
- Budget or explanation of how resources will made available to undertake the research project.
The AIPFR PIC will be review and consider the full application as they are received.
Successful researchers will be asked to help AILDR Manager facilitate signing of a “Release Information Agreement for research projects” between University of Sydney and their institution.
If the research will be undertaken at or by a Principal Investigator located by an institution that is not part of the National Mutual Acceptance (NMA) Scheme for ethics review, the researchers will also need to make a Site Specific Application (SSA) to the Sydney Local Health District (RPAH Zone) HREC.
A fee to cover the preparation of AIPFR data may be charged.
Publications of research using AIPFR data will be required adhere to the AIPFR Publication policy V1.4 4 Sept 2022.
Humphries SM, Mackintosh JA, Jo HE, et al. Quantitative computed tomography predicts outcomes in idiopathic pulmonary fibrosis [published online ahead of print, 2022 Jul 25]. Respirology. 2022;10.1111/resp.14333. doi:10.1111/resp.14333
Cox IA, Campbell J, de Graaff B, et al. Assessment of health-related quality of life in Australian patients with idiopathic pulmonary fibrosis: a comparison of the EQ-5D-5L and the AQoL-8D [published online ahead of print, 2022 Aug 4]. Qual Life Res. 2022;10.1007/s11136-022-03205-z. doi:10.1007/s11136-022-03205-z
Walsh SL, Mackintosh JA, Calandriello L, et al. Deep Learning-based Outcome Prediction in Progressive Fibrotic Lung Disease Using High-resolution Computed Tomography [published online ahead of print, 2022 Jun 13]. Am J Respir Crit Care Med. 2022;10.1164/rccm.202112-2684OC. doi:10.1164/rccm.202112-2684OC
Clynick B, Corte TJ, Jo HE, et al. Biomarker signatures for progressive idiopathic pulmonary fibrosis. Eur Respir J. 2022;59(3):2101181. Published 2022 Mar 31. doi:10.1183/13993003.01181-2021
Cox IA, Otahal P, de Graaff B, et al. Incidence, prevalence and mortality of idiopathic pulmonary fibrosis in Australia. Respirology. 2022;27(3):209-216. doi:10.1111/resp.14194
Tikellis G, Corte TJ, Teoh AKY, et al. Barriers and facilitators to best care for idiopathic pulmonary fibrosis in Australia Respirology. 2021 DOI: 10.1111/resp.14185
Zhao A, Gudmundsson E, Mogulkoc N, et al. Mortality in combined pulmonary fibrosis and emphysema patients is determined by the sum of pulmonary fibrosis and emphysema. ERJ Open Res. 2021;7(3):00316-2021. Published 2021 Aug 23. doi:10.1183/23120541.00316-2021
Cox IA, de Graaff B, Ahmed H, et al. The impact of idiopathic pulmonary fibrosis on health state utility values: evidence from Australia. Qual Life Res. 2021;30(9):2615-2632. doi:10.1007/s11136-021-02879-1
Nambiar S, Clynick B, How BS, et al. There is detectable variation in the lipidomic profile between stable and progressive patients with idiopathic pulmonary fibrosis (IPF). Respir Res. 2021;22(1):105. Published 2021 Apr 9. doi:10.1186/s12931-021-01682-3
Tikellis G, Tong A, Lee JYT, et al. Top 10 research priorities for people living with pulmonary fibrosis, their caregivers, healthcare professionals and researchers. Thorax. 2021;76(6):575-581. doi:10.1136/thoraxjnl-2020-215731
Baumgartner KB, Samet JM, Coultas DB, et al. Occupational and environmental risk factors for idiopathic pulmonary fibrosis: a multicenter case-control study. Collaborating Centers. Am J Epidemiol. 2000;152(4):307-315. doi:10.1093/aje/152.4.307
Teoh AKY, Glaspole I, Macansh S, Corte TJ. Importance of Occupational Exposure Data: A National Idiopathic Pulmonary Fibrosis Registry Perspective. Am J Respir Crit Care Med. 2020;201(9):1165-1167. doi:10.1164/rccm.201911-2242LE
Clynick B, Jo HE, Corte TJ, et al. Circulating RNA differences between patients with stable and progressive idiopathic pulmonary fibrosis. Eur Respir J. 2020;56(3):1902058. Published 2020 Sep 10. doi:10.1183/13993003.02058-2019
Teoh AKY, Jo HE, Chambers DC, et al. Blood monocyte counts as a potential prognostic marker for idiopathic pulmonary fibrosis: analysis from the Australian IPF registry. Eur Respir J. 2020;55(4):1901855. Published 2020 Apr 3. doi:10.1183/13993003.01855-2019
Moodley YP, Corte TJ, Oliver BG, et al. Analysis by proteomics reveals unique circulatory proteins in idiopathic pulmonary fibrosis. Respirology. 2019;24(11):1111-1114. doi:10.1111/resp.13668
Jo, H.E., Corte, T.J., Glaspole, I. et al. Gastroesophageal reflux and antacid therapy in IPF: analysis from the Australia IPF Registry. BMC Pulm Med 19, 84 (2019). https://doi.org/10.1186/s12890-019-0846-2
Burgess A, Goon K, Brannan JD, et al. Eligibility for anti-fibrotic treatment in idiopathic pulmonary fibrosis depends on the predictive equation used for pulmonary function testing. Respirology. 2019;24(10):988-995. doi:10.1111/resp.13540
Jo HE, Glaspole I, Goh N, et al. Implications of the diagnostic criteria of idiopathic pulmonary fibrosis in clinical practice: Analysis from the Australian Idiopathic Pulmonary Fibrosis Registry. Respirology. 2019;24(4):361-368. doi:10.1111/resp.13427
Jo HE, Glaspole I, Moodley Y, et al. Disease progression in idiopathic pulmonary fibrosis with mild physiological impairment: analysis from the Australian IPF registry. BMC Pulm Med. 2018;18(1):19. Published 2018 Jan 25. doi:10.1186/s12890-018-0575-y
Glaspole IN, Watson AL, Allan H, et al. Determinants and outcomes of prolonged anxiety and depression in idiopathic pulmonary fibrosis. Eur Respir J. 2017;50(2):1700168. Published 2017 Aug 17. doi:10.1183/13993003.00168-2017
Glaspole IN, Chapman SA, Cooper WA, et al. Health-related quality of life in idiopathic pulmonary fibrosis: Data from the Australian IPF Registry. Respirology. 2017;22(5):950-956. doi:10.1111/resp.12989
Jo HE, Glaspole I, Grainge C, et al. Baseline characteristics of idiopathic pulmonary fibrosis: analysis from the Australian Idiopathic Pulmonary Fibrosis Registry Eur Respir J. 2017 Mar 29;49(3). doi:10.1183/13993003.01592-2016
Troy LK, Chapman SA, Lake F, et al. Current Australasian practice for diagnosis and management of idiopathic pulmonary fibrosis: Where are we now?. Respirology. 2015;20(4):647-653. doi:10.1111/resp.12512*
Moodley Y, Goh N, Glaspole I, et al. Australian Idiopathic Pulmonary Fibrosis Registry: vital lessons from a national prospective collaborative project. Respirology. 2014;19(7):1088-1091. doi:10.1111/resp.12358
*Does not use AIPFR data but involved the AIPFR team
