Interstitial Lung Disease (ILD)INDEXNCT06685874
Interstitial Lung Disease Exacerbations Study
An observational study of Interstitial Lung Disease (ILD) and Pulmonary Fibrosis, run by Royal Devon and Exeter NHS Foundation Trust at 3 sites in United Kingdom.
Trial summary
Interstitial lung disease (ILD) is an umbrella term covering numerous conditions that affect the lung tissue, interfering with the ability of the lungs to take up oxygen. Most ILDs get worse gradually, but sometimes patients can experience a sudden worsening in their symptoms called an acute exacerbation (AE-ILD). Read more about this study
- Who is it for?
- Ages 18 years and older
- Conditions the study is for
- Interstitial Lung Disease (ILD), Pulmonary Fibrosis
- How many people
- 1,500 participants
- Where
- 3 sitesUnited Kingdom
- Study dates
- 1 May 2025to 1 Aug 2026
- Phase
- Observational
- Are there results?
- Not yet: the study is under way
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 18 years and older
- All sexes
- No healthy volunteers
- Adult, Older adult
Study population: Patients with fibrotic interstitial lung disease admitted to hospital with exacerbation of pulmonary fibrosis as per inclusion and exclusion criteria
Who may be able to join
- Patients admitted to an acute NHS trust during study period 1st September 2022-31st August 2023 with an underlying or new diagnosis of an ILD (defined by ICD-10 codes in Table 1) with a fibrotic phenotype (established fibrosis on current or previous radiological imaging) AND
- Increasing pulmonary symptoms AND
- No extra-parenchymal cause identified as primary cause of admission
- Segmental or larger pulmonary embolus as main discharge diagnosis in absence of acute exacerbation features on imaging
- Pleural effusion as main discharge diagnosis
- Pneumothorax as main discharge diagnosis in absence of acute exacerbation features on imaging
- Left sided heart failure or volume overload
Who cannot join
- No underlying or new diagnosis of ILD with a fibrotic phenotype
- Acute presentation of pneumonitis/ILD without evidence of fibrosis (Table 2)
- Non-respiratory related hospital admission (no increasing pulmonary symptoms)
- Respiratory related hospital admission due to extra-parenchymal cause
- Segmental or larger pulmonary embolus as main discharge diagnosis in absence of acute exacerbation features on imaging
- Pleural effusion as main discharge diagnosis
Show all 12Show fewer
- Pneumothorax as main discharge diagnosis in absence of acute exacerbation features on imaging
- Left sided heart failure or volume overload
- Participant signatory to NHS National Data Opt Out
- Elective/non-emergency admission
- Admission under non-medical specialty
- In cases where there are >=1 eligible admissions only the first (index) admission shall be included
Trial locations and contacts
3 sites in 1 country.
United Kingdom3 sites
Royal United Hospitals Bath NHS Foundation Trust
Bath
North Bristol NHS Trust
Bristol
Royal Devon University Hospitals NHS Foundation Trust
Exeter
About this study
Interstitial lung disease (ILD) is an umbrella term covering numerous conditions that affect the lung tissue, interfering with the ability of the lungs to take up oxygen. Most ILDs get worse gradually, but sometimes patients can experience a sudden worsening in their symptoms called an acute exacerbation (AE-ILD). Most studies in this area have been done in AEs of idiopathic pulmonary fibrosis (AE-IPF), as IPF is the commonest form of ILD. AE-IPF has very poor outcomes, however AEs of other ILDs are less well studied. Furthermore, there is currently no treatment guideline or established standard of care for the management of patients with AE-fILD.
The aim of this research project is to gain a better understanding of AE-ILD in a real-world population. By looking at the clinical records of patients with AE-ILD, the study aims to describe the patient population that gets AE-ILD and how these patients are treated in the "real world" setting. The study will also gather information on patient characteristics such as type of ILD and test results at the time of AE-ILD, and see if any of these factors are associated with better/ worse outcomes in AE-ILD. Finally, the study will collect data on the treatment approaches taken, including both medical therapy such as steroid treatment, as well as specialist care team input. This data on treatment will be used to identify associations between individual treatments and outcomes, as well as to evaluate the NHS services being provided to patients with AE-ILD.
Overall, this study will enhance understanding of AE-ILD. This study will provide information to help design clinical trials to test treatments for AE-ILD, to help us create evidence-based clinical guidelines for AE-ILD, and improve the management of patients with AE-ILD.
Read the detailed description
Idiopathic pulmonary fibrosis (IPF) and other progressive fibrotic interstitial lung diseases (PF-ILDs) are increasingly common fatal disorders with a median life expectancy from diagnosis of 3-5 years. Fibrotic interstitial lung disease (fILD) is a general term for a group of conditions that involve scarring of the lung (fibrosis) with or without evidence of inflammation. While different ILDs are associated with different aetiological factors and disease courses, there is considerable overlap in the characteristics of these conditions. In general, fILDs usually progress in terms of symptoms, lung function, and radiological features, resulting in increasing symptoms and respiratory failure.
In addition to the gradual progression that is typical of fILD, these conditions can become acutely worse during episodes of acute exacerbation (AE-fILD). An AE can be defined as an acute (less than one month) deterioration in symptoms associated with new ground glass changes on CT chest and not explained by other pathology such as pulmonary embolus, pneumothorax, or cardiac failure (adapted from the definition by Collard et al for AE-IPF). The prognosis of acute exacerbations of idiopathic pulmonary fibrosis (IPF), the commonest form of fILD, is very poor (over 90% in-hospital mortality in some reports). However, there has been very little research on AEs in non-IPF fILD.
Furthermore, there is currently no treatment guideline or established standard of care for the management of patients with AE-fILD. Other significant gaps in the literature in this area, include:
- What is the typical clinical course and outcome of acute exacerbations in non-IPF fILD?
- What features predict the risk of in-hospital and post-discharge mortality from AE-fILD (e.g. age, sex, baseline diagnosis)?
- Are currently used treatments for AE-fILD effective? How should AE-fILD be managed (steroids, oxygen therapy etc)?
- How well are aspects of care that patients find important (e.g. provision of specialist palliative care, opportunities to discuss advanced care planning, and oxygen therapy) delivered to patients with AE-fILD?
There are significant gaps in the literature in the field of AE-fILD. Much of the current practice in AE-fILD management is based on clinical judgement, and adequately powered randomised controlled trials to identify appropriate treatment approaches have not been performed. This has led to significant heterogeneity in the approach to AE-fILD between clinicians and centres, an issue illustrated by the fact that only one of seven NHS trusts in the East Midlands has a treatment guideline specific for the management of AE-fILD (unpublished data). This lack of consistency in approach poses a significant challenge to the design of randomised control trials, as heterogeneity within a standard care control arm can obscure any specific signals. Furthermore, in the absence of RCTs, there is the potential that clinicians may unintentionally use interventions that are harmful.
The study is an observational cohort study of patients admitted to hospital with AE-fILD to answer the questions listed above. These data will help to clarify research priorities in this area, and identify areas where care could improve and allow us to make recommendations for clinical care. The study will also use these data to inform and generate hypotheses for future clinical trials.
- Keywords
- Interstitial lung disease, Exacerbation
- Medical subject headings
- Lung Diseases, Interstitial, Pulmonary Fibrosis
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
| Study group | Role | What they receive |
|---|---|---|
| Patients with exacerbation of fibrotic interstitial lung disease | Patients with exacerbation of fibrotic interstitial lung disease as per inclusion and exclusion criteria |
What is being measured
The primary outcome is the main question the study is designed to answer.
Transplant free survival
Transplant-free survival
Measured: 90 days post date of hospital admission
Secondary outcomes (4)
Transplant free survival
Transplant free survival
Measured: Date of discharge from hospital for index admission (Up to 24 hours), 30 days, 90 days (Primary endpoint), 6 months, 1 year
Pulmonary Function Testing
Change in forced vital capacity (FVC) from pre-admission values
Measured: 6 months and 12 months from date of index admission
Pulmonary function testing
Change in diffusion capacity (DLCO) from pre-admission values
Measured: 6 months and 12 months from date of index admission
Oxygen
Use of long term or ambulatory oxygen
Measured: At the point of discharge from index admission (up to 24 hours)
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted13 Nov 2024
- Study start1 May 2025
- Primary completion1 Aug 2026 (estimated)
- Study completion1 Aug 2026 (estimated)
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Publications (1)Papers the sponsor has linked to this study.
- Other trials for Interstitial Lung Disease (ILD)Studies of the same condition, open and under way.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Observational
- Observational model
- Cohort
- Time perspective
- Retrospective
- Enrolment
- 1,500 participants (estimated)
Sponsor and investigators
- Lead sponsor
- Royal Devon and Exeter NHS Foundation Trust
- Sponsor type
- University, hospital or other
- Collaborators
- University Hospitals, Leicester, Nottingham University Hospitals NHS Trust, North Bristol NHS Trust, Papworth Hospital NHS Foundation Trust
- Responsible party
- Sponsor
- Investigators
- Michael Gibbons, MBChB, Principal investigator, Royal Devon and University healthcare trust
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT06685874
- Sponsor's protocol ID
- 2501703
- Acronym
- INDEX
- Status last verified
- Apr 2025
- First submitted
- 30 Oct 2024
- Last updated
- 18 May 2025
- Results posted
- No
- Data monitoring committee
- No
- FDA-regulated drug
- No
- FDA-regulated device
- No
- Individual data sharing
- No: IPD will not be shared due to lack of informed consent.
Publications
Goodwin AT, Dixon G, White LJ, Virk H, Nadeem I, Saini G, Khan F, Parfrey H, Scotton CJ, Barratt SL, Taylor G, Gibbons M. Protocol for the INterstitial lung Disease EXacerbations (INDEX) Study: a retrospective multicentre cohort study. BMJ Open Respir Res. 2026 Jun 9;13(1):e003949. doi: 10.1136/bmjresp-2025-003949.
PubMed 42264883
Study at a glance
From the registry record, last updated by the sponsor on 18 May 2025. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Interstitial Lung Disease Exacerbations Study
- ClinicalTrials.gov ID
- NCT06685874
- Acronym / sponsor ID
- INDEX / 2501703
- Keywords
- Interstitial lung disease, Exacerbation
- Study type
- Observational
- Recruitment status
- Active, not recruiting
- Enrolment
- 1,500 participants (estimated)
- Lead sponsor
- Royal Devon and Exeter NHS Foundation TrustUniversity, hospital or other
- Collaborators
- University Hospitals, Leicester, Nottingham University Hospitals NHS Trust, North Bristol NHS Trust, Papworth Hospital NHS Foundation Trust
- Study start
- 1 May 2025 (actual)
- Primary completion
- 1 Aug 2026 (estimated)
- Study completion
- 1 Aug 2026 (estimated)
- First posted
- 13 Nov 2024 (actual)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
Interstitial lung disease (ILD) is an umbrella term covering numerous conditions that affect the lung tissue, interfering with the ability of the lungs to take up oxygen. Most ILDs get worse gradually, but sometimes patients can experience a sudden worsening in their symptoms called an acute exacerbation (AE-ILD). Most studies in this area have been done in AEs of idiopathic pulmonary fibrosis (AE-IPF), as IPF is the commonest form of ILD. AE-IPF has very poor outcomes, however AEs of other ILDs are less well studied. Furthermore, there is currently no treatment guideline or established standard of care for the management of patients with AE-fILD.
The aim of this research project is to gain a better understanding of AE-ILD in a real-world population. By looking at the clinical records of patients with AE-ILD, the study aims to describe the patient population that gets AE-ILD and how these patients are treated in the "real world" setting. The study will also gather information on patient characteristics such as type of ILD and test results at the time of AE-ILD, and see if any of these factors are associated with better/ worse outcomes in AE-ILD. Finally, the study will collect data on the treatment approaches taken, including both medical therapy such as steroid treatment, as well as specialist care team input. This data on treatment will be used to identify associations between individual treatments and outcomes, as well as to evaluate the NHS services being provided to patients with AE-ILD.
Overall, this study will enhance understanding of AE-ILD. This study will provide information to help design clinical trials to test treatments for AE-ILD, to help us create evidence-based clinical guidelines for AE-ILD, and improve the management of patients with AE-ILD.
Detailed description
Idiopathic pulmonary fibrosis (IPF) and other progressive fibrotic interstitial lung diseases (PF-ILDs) are increasingly common fatal disorders with a median life expectancy from diagnosis of 3-5 years. Fibrotic interstitial lung disease (fILD) is a general term for a group of conditions that involve scarring of the lung (fibrosis) with or without evidence of inflammation. While different ILDs are associated with different aetiological factors and disease courses, there is considerable overlap in the characteristics of these conditions. In general, fILDs usually progress in terms of symptoms, lung function, and radiological features, resulting in increasing symptoms and respiratory failure.
In addition to the gradual progression that is typical of fILD, these conditions can become acutely worse during episodes of acute exacerbation (AE-fILD). An AE can be defined as an acute (less than one month) deterioration in symptoms associated with new ground glass changes on CT chest and not explained by other pathology such as pulmonary embolus, pneumothorax, or cardiac failure (adapted from the definition by Collard et al for AE-IPF). The prognosis of acute exacerbations of idiopathic pulmonary fibrosis (IPF), the commonest form of fILD, is very poor (over 90% in-hospital mortality in some reports). However, there has been very little research on AEs in non-IPF fILD.
Furthermore, there is currently no treatment guideline or established standard of care for the management of patients with AE-fILD. Other significant gaps in the literature in this area, include:
- What is the typical clinical course and outcome of acute exacerbations in non-IPF fILD?
- What features predict the risk of in-hospital and post-discharge mortality from AE-fILD (e.g. age, sex, baseline diagnosis)?
- Are currently used treatments for AE-fILD effective? How should AE-fILD be managed (steroids, oxygen therapy etc)?
- How well are aspects of care that patients find important (e.g. provision of specialist palliative care, opportunities to discuss advanced care planning, and oxygen therapy) delivered to patients with AE-fILD?
There are significant gaps in the literature in the field of AE-fILD. Much of the current practice in AE-fILD management is based on clinical judgement, and adequately powered randomised controlled trials to identify appropriate treatment approaches have not been performed. This has led to significant heterogeneity in the approach to AE-fILD between clinicians and centres, an issue illustrated by the fact that only one of seven NHS trusts in the East Midlands has a treatment guideline specific for the management of AE-fILD (unpublished data). This lack of consistency in approach poses a significant challenge to the design of randomised control trials, as heterogeneity within a standard care control arm can obscure any specific signals. Furthermore, in the absence of RCTs, there is the potential that clinicians may unintentionally use interventions that are harmful.
The study is an observational cohort study of patients admitted to hospital with AE-fILD to answer the questions listed above. These data will help to clarify research priorities in this area, and identify areas where care could improve and allow us to make recommendations for clinical care. The study will also use these data to inform and generate hypotheses for future clinical trials.
Study design
- Observational model
- Cohort
- Time perspective
- Retrospective
- Number of arms
- 1
| Arm | Type | Receives |
|---|---|---|
| Patients with exacerbation of fibrotic interstitial lung disease | – | –Patients with exacerbation of fibrotic interstitial lung disease as per inclusion and exclusion criteria |
Who may qualify
- Ages
- 18 years and older
- Sex
- All
- Healthy volunteers
- Not accepted
- Age groups
- Adult, Older adult
- Study population
- Patients with fibrotic interstitial lung disease admitted to hospital with exacerbation of pulmonary fibrosis as per inclusion and exclusion criteria
Key inclusion criteria (7 listed)
- Patients admitted to an acute NHS trust during study period 1st September 2022-31st August 2023 with an underlying or new diagnosis of an ILD (defined by ICD-10 codes in Table 1) with a fibrotic phenotype (established fibrosis on current or previous radiological imaging) AND
- Increasing pulmonary symptoms AND
- No extra-parenchymal cause identified as primary cause of admission
Key exclusion criteria (12 listed)
- No underlying or new diagnosis of ILD with a fibrotic phenotype
- Acute presentation of pneumonitis/ILD without evidence of fibrosis (Table 2)
- Non-respiratory related hospital admission (no increasing pulmonary symptoms)
- Respiratory related hospital admission due to extra-parenchymal cause
- Participant signatory to NHS National Data Opt Out
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Primary outcome
Transplant free survival
Time frame: 90 days post date of hospital admission
Transplant-free survival
4 secondary outcomes
Transplant free survival
Time frame: Date of discharge from hospital for index admission (Up to 24 hours), 30 days, 90 days (Primary endpoint), 6 months, 1 year
Pulmonary Function Testing
Time frame: 6 months and 12 months from date of index admission
Pulmonary function testing
Time frame: 6 months and 12 months from date of index admission
Oxygen
Time frame: At the point of discharge from index admission (up to 24 hours)
Results and safety
This study is still under way. Results are not available in the source record yet.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 18 May 2025.
- Derived from this study: PubMed 42264883Goodwin AT, Dixon G, White LJ, Virk H, Nadeem I, Saini G, Khan F, Parfrey H, Scotton CJ, Barratt SL, Taylor G, Gibbons M. Protocol for the INterstitial lung Disease EXacerbations (INDEX) Study: a retrospective multicentre cohort study. BMJ Open Respir Res. 2026 Jun 9;13(1):e003949. doi: 10.1136/bmjresp-2025-003949.
- Individual participant dataSharing planned: No. IPD will not be shared due to lack of informed consent.
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Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part. This study is not recruiting at the moment.
Michael Gibbons, MBChB principal investigator
Royal Devon and University healthcare trust
3 sites in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
An observational study of Interstitial Lung Disease (ILD) and Pulmonary Fibrosis, run by Royal Devon and Exeter NHS Foundation Trust at 3 sites in United Kingdom.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 7 inclusion and 12 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: under way, not looking for participants, as of the sponsor's last update on 18 May 2025. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 3 sites in United Kingdom. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
This study is still under way. Results are not available in the source record yet.
How can a doctor refer a patient or contact the research team?
The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
Transplant free survival (time frame: 90 days post date of hospital admission). It is what the study was designed to measure, not a finding.
Where can I read the original study information?
On the ClinicalTrials.gov record for NCT06685874, which also lists 1 linked publication.
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 18 May 2025.