Pulmonary Fibrosis Idiopathic FamilialFLORISNCT05367349
Optimising Screening for Early Disease Detection in Familial Pulmonary Fibrosis
An observational study of Pulmonary Fibrosis Idiopathic Familial, run by St. Antonius Hospital at 1 site in Netherlands.
Trial summary
In this study the prognostic value of the current screening parameters for familial pulmonary fibrosis (FPF) will be investigated by looking at the screenings of 200 first-degree relatives of patients with FPF. Read more about this study
- Who is it for?
- Ages 18 years and older
- Conditions the study is for
- Pulmonary Fibrosis Idiopathic Familial
- How many people
- 200 participants
- Where
- 1 siteNetherlands
- Study dates
- 16 Jun 2021to 1 Sep 2025
- Phase
- Observational
- Are there results?
- None posted in the registry
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: Asymptomatic first-degree relatives of patients with FPF are invited to enroll.
Who may be able to join
- Asymptomatic first-degree relative of patients with familial pulmonary fibrosis (FPF)
Who cannot join
- A previous diagnosis of interstitial lung disease (ILD)
- Minors (aged <18 years)
- Pregnant
- Note: woman who are pregnant at the start of the study or at the time of the HRCT are not allowed to participate. If a participant gets pregnant at a later stage during the study, she will not be excluded from the study. To be able to account for a potential effect of pregnancy during data analysis, female participants can be asked if they are pregnant at every visit.
Trial locations and contacts
1 site in 1 country.
Netherlands1 site, 1 recruiting
- Recruiting
St Antonius Hospital
Nieuwegein, 3435CM
Jan Grutters, Prof., +31883201453, j.grutters@antoniusziekenhuis.nl
Martijn Maus, MD, +31883201467, m.maus@antoniusziekenhuis.nl
About this study
In this study the prognostic value of the current screening parameters for familial pulmonary fibrosis (FPF) will be investigated by looking at the screenings of 200 first-degree relatives of patients with FPF.
Also insight in the natural history of early FPF, and the necessary interval between screenings visits will be investigated.
Read the detailed description
Familial pulmonary fibrosis (FPF) is a fatal lung disease that is often not diagnosed until a significant portion of the lung function is lost. Median survival after diagnosis is 3 to 5 years. As treatment can only slow down lung function decline, early disease detection is essential to provide timely therapeutic support. As first-degree relatives of patients with FPF are at high risk of developing pulmonary fibrosis as well, a screening protocol has been put in place. However, the value of current screening parameters to detect early asymptomatic disease as well as the optimal interval between screening appointments are unknown. A prospective study into the prognostic value of these screening markers in the target population and the appropriate clinical setting is needed to develop an evidence-based screening protocol. There will be an emphasis on easily operable parameters that may allow for redirection of (part of the) screening activities to the general practice in the future.
- Conditions
- Pulmonary Fibrosis Idiopathic Familial
- Keywords
- Pulmonary Fibrosis, Familial, Screening, Asymptomatic, Family members, First-degree
- Medical subject headings
- Idiopathic Pulmonary Fibrosis, Pulmonary Fibrosis
What is being measured
The primary outcome is the main question the study is designed to answer.
Characteristics of participants
Difference in age, sex, body weight, smoking history and comorbidities between the group with ILD changes and the group without ILD changes present on enrollment HRCT
Measured: Baseline
Forced Vital Capacity (FVC)
Difference in forced vital capacity (FVC) between the group with ILD changes and the group without ILD changes present on enrollment HRCT
Measured: Baseline
Haemoglobulin-corrected carbon monoxide diffusing capacity (DLCOc)
Difference in haemoglobulin-corrected carbon monoxide diffusing capacity (DLCOc) between the group with ILD changes and the group without ILD changes present on enrollment HRCT
Measured: Baseline
Total lung capacity (TLC)
Difference in total lung capacity (TLC) between the group with ILD changes and the group without ILD changes present on enrollment HRCT
Measured: Baseline
Biomarker levels
Difference in Krebs von den Lungen 6 (KL6), chemokine (C-C motif) ligand 18 (CCL18), surfactant protein-D (SP-D), matrix metalloproteinase 7 (MMP7) between the group with ILD changes and the group without ILD changes present on enrollment HRCT
Measured: Baseline
Oxygen saturation in 6-minute walk test (6MWT)
Difference in oxygen saturation (rest in %), oxygen saturation (after 6MWT in %), oxygen saturation (nadir in %) and oxygen saturation (difference between rest and nadir in %) (all continuous variables) between the group with ILD changes and the group without ILD changes present on enrollment HRCT
Measured: Baseline
Distance (meters) in 6-minute walk test (6MWT)
Difference in distance covered (in meters) between the group with ILD changes and the group without ILD changes present on enrollment HRCT
Measured: Baseline
MUC5B genotype
Difference in MUC5B genotype (all discrete variables) between the group with ILD changes and the group without ILD changes present on enrollment HRCT
Measured: Baseline
Patient reported cough score
Difference in visual analogue scale (VAS) for cough (scores between 0-100, with 100 being the worst) between the group with ILD changes and the group without ILD changes present on enrollment HRCT
Measured: Baseline
Patient reported dyspnea score
Difference in medical research council (MRC) (scores between 0-5, with 5 being the worst) between the group with ILD changes and the group without ILD changes present on enrollment HRCT
Measured: Baseline
Clubbing
Difference in presence of digital clubbing between the group with ILD changes and the group without ILD changes present on enrollment HRCT
Measured: Baseline
Crackles
Difference in inspiratory crackles during lung auscultation between the group with ILD changes and the group without ILD changes present on enrollment HRCT
Measured: Baseline
Secondary outcomes (13)
Forced Vital Capacity (FVC)
Difference in forced vital capacity (FVC) over the course of 2 years in the subjects with presence of ILD changes on enrollment HRCT and also between the group with ILD changes and the group without ILD changes present on enrollment HRCT
Measured: 2 years
Haemoglobulin-corrected carbon monoxide diffusing capacity (DLCOc)
Difference in haemoglobulin-corrected carbon monoxide diffusing capacity (DLCOc) over the course of 2 years in the subjects with presence of ILD changes on enrollment HRCT and also between the group with ILD changes and the group without ILD changes present on enrollment HRCT
Measured: 2 years
Total lung capacity (TLC)
Difference in total lung capacity (TLC) over the course of 2 years in the subjects with presence of ILD changes on enrollment HRCT and also between the group with ILD changes and the group without ILD changes present on enrollment HRCT
Measured: 2 years
Biomarker levels
Difference in KL6, CCL18, SP-D and MMP7 over the course of 2 years in the subjects with presence of ILD changes on enrollment HRCT and also between the group with ILD changes and the group without ILD changes present on enrollment HRCT
Measured: 2 years
Genotype
Difference in MUC5B genotype over the course of 2 years in the subjects with presence of ILD changes on enrollment HRCT and also between the group with ILD changes and the group without ILD changes present on enrollment HRCT
Measured: 2 years
Oxygen saturation in 6-minute walk test (6MWT)
Difference in oxygen saturation (rest in %), oxygen saturation (after 6MWT in %), oxygen saturation (nadir in %) and oxygen saturation (difference between rest and nadir in %) (all continuous variables) over the course of 2 years in the subjects with presence of ILD changes on enrollment HRCT and also between the group with ILD changes and the group without ILD changes present on enrollment HRCT
Measured: 2 years
Distance (meters) in 6-minute walk test (6MWT)
Difference in distance covered (in meters) over the course of 2 years in the subjects with presence of ILD changes on enrollment HRCT and also between the group with ILD changes and the group without ILD changes present on enrollment HRCT
Measured: 2 years
Patient reported cough score
Difference in visual analogue scale (VAS) for cough (scores between 0-100, with 100 being the worst) over the course of 2 years in the subjects with presence of ILD changes on enrollment HRCT and also between the group with ILD changes and the group without ILD changes present on enrollment HRCT
Measured: 2 years
Patient reported dyspnea score
Difference in medical research council (MRC) (scores between 0-5, with 5 being the worst) over the course of 2 years in the subjects with presence of ILD changes on enrollment HRCT and also between the group with ILD changes and the group without ILD changes present on enrollment HRCT
Measured: 2 years
Digital clubbing
Difference in presence of digital clubbing over the course of 2 years in the subjects with presence of ILD changes on enrollment HRCT and also between the group with ILD changes and the group without ILD changes present on enrollment HRCT
Measured: 2 years
Crackles
Difference in inspiratory crackles during lung auscultation over the course of 2 years in the subjects with presence of ILD changes on enrollment HRCT and also between the group with ILD changes and the group without ILD changes present on enrollment HRCT
Measured: 2 years
Patient reported fatigue
Difference in fatigue assessment scale (FAS) (scores range from 10-50, with 50 being the worst) over the course of 2 years in the subjects with presence of ILD changes on enrollment HRCT and also between the group with ILD changes and the group without ILD changes present on enrollment HRCT
Measured: 2 years
Patient reported health status
Difference in EuroQol 5D-5L (EQ-5D-5L) (comprises of a score in five levels, level 1-5 with 5 being the worst, and a visual analoque scale ranging from 0-100 with 100 being the best) and the over the course of 2 years in the subjects with presence of ILD changes on enrollment HRCT and also between the group with ILD changes and the group without ILD changes present on enrollment HRCT
Measured: 2 years
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted10 May 2022
- Study start16 Jun 2021
- Primary completion16 Jun 2025 (estimated)
- Study completion1 Sep 2025 (estimated)
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Other trials for Pulmonary Fibrosis Idiopathic FamilialStudies 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
- Prospective
- Enrolment
- 200 participants (estimated)
Sponsor and investigators
- Lead sponsor
- St. Antonius Hospital
- Sponsor type
- University, hospital or other
- Collaborators
- ZonMw: The Netherlands Organisation for Health Research and Development, Boehringer Ingelheim
- Responsible party
- Principal investigator, Jan C. Grutters, MD, St. Antonius Hospital
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT05367349
- Sponsor's protocol ID
- NL75303.100.20
- Acronym
- FLORIS
- Status last verified
- Mar 2022
- First submitted
- 28 Mar 2022
- Last updated
- 10 May 2022
- Results posted
- No
- Data monitoring committee
- No
- FDA-regulated drug
- No
- FDA-regulated device
- No
- Individual data sharing
- No: Data of patients is coded
Study at a glance
From the registry record, last updated by the sponsor on 10 May 2022. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Optimising Screening for Early Disease Detection in Familial Pulmonary Fibrosis
- ClinicalTrials.gov ID
- NCT05367349
- Acronym / sponsor ID
- FLORIS / NL75303.100.20
- Conditions
- Pulmonary Fibrosis Idiopathic Familial
- Keywords
- Pulmonary Fibrosis, Familial, Screening, Asymptomatic, Family members, First-degree
- Study type
- Observational
- Recruitment status
- Unknown status
- Enrolment
- 200 participants (estimated)
- Lead sponsor
- St. Antonius HospitalUniversity, hospital or other
- Collaborators
- ZonMw: The Netherlands Organisation for Health Research and Development, Boehringer Ingelheim
- Study start
- 16 Jun 2021 (actual)
- Primary completion
- 16 Jun 2025 (estimated)
- Study completion
- 1 Sep 2025 (estimated)
- First posted
- 10 May 2022 (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.
In this study the prognostic value of the current screening parameters for familial pulmonary fibrosis (FPF) will be investigated by looking at the screenings of 200 first-degree relatives of patients with FPF.
Also insight in the natural history of early FPF, and the necessary interval between screenings visits will be investigated.
Detailed description
Familial pulmonary fibrosis (FPF) is a fatal lung disease that is often not diagnosed until a significant portion of the lung function is lost. Median survival after diagnosis is 3 to 5 years. As treatment can only slow down lung function decline, early disease detection is essential to provide timely therapeutic support. As first-degree relatives of patients with FPF are at high risk of developing pulmonary fibrosis as well, a screening protocol has been put in place. However, the value of current screening parameters to detect early asymptomatic disease as well as the optimal interval between screening appointments are unknown. A prospective study into the prognostic value of these screening markers in the target population and the appropriate clinical setting is needed to develop an evidence-based screening protocol. There will be an emphasis on easily operable parameters that may allow for redirection of (part of the) screening activities to the general practice in the future.
Study design
- Observational model
- Cohort
- Time perspective
- Prospective
Who may qualify
- Ages
- 18 years and older
- Sex
- All
- Healthy volunteers
- Not accepted
- Age groups
- Adult, Older adult
- Study population
- Asymptomatic first-degree relatives of patients with FPF are invited to enroll.
Key inclusion criteria (1 listed)
- Asymptomatic first-degree relative of patients with familial pulmonary fibrosis (FPF)
Key exclusion criteria (4 listed)
- A previous diagnosis of interstitial lung disease (ILD)
- Minors (aged <18 years)
- Pregnant
- Note: woman who are pregnant at the start of the study or at the time of the HRCT are not allowed to participate. If a participant gets pregnant at a later stage during the study, she will not be excluded from the study. To be able to account for a potential effect of pregnancy during data analysis, female participants can be asked if they are pregnant at every visit.
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
Characteristics of participants
Time frame: Baseline
Difference in age, sex, body weight, smoking history and comorbidities between the group with ILD changes and the group without ILD changes present on enrollment HRCT
Forced Vital Capacity (FVC)
Time frame: Baseline
Difference in forced vital capacity (FVC) between the group with ILD changes and the group without ILD changes present on enrollment HRCT
Haemoglobulin-corrected carbon monoxide diffusing capacity (DLCOc)
Time frame: Baseline
Difference in haemoglobulin-corrected carbon monoxide diffusing capacity (DLCOc) between the group with ILD changes and the group without ILD changes present on enrollment HRCT
Total lung capacity (TLC)
Time frame: Baseline
Difference in total lung capacity (TLC) between the group with ILD changes and the group without ILD changes present on enrollment HRCT
Biomarker levels
Time frame: Baseline
Difference in Krebs von den Lungen 6 (KL6), chemokine (C-C motif) ligand 18 (CCL18), surfactant protein-D (SP-D), matrix metalloproteinase 7 (MMP7) between the group with ILD changes and the group without ILD changes present on enrollment HRCT
Oxygen saturation in 6-minute walk test (6MWT)
Time frame: Baseline
Difference in oxygen saturation (rest in %), oxygen saturation (after 6MWT in %), oxygen saturation (nadir in %) and oxygen saturation (difference between rest and nadir in %) (all continuous variables) between the group with ILD changes and the group without ILD changes present on enrollment HRCT
Distance (meters) in 6-minute walk test (6MWT)
Time frame: Baseline
Difference in distance covered (in meters) between the group with ILD changes and the group without ILD changes present on enrollment HRCT
MUC5B genotype
Time frame: Baseline
Difference in MUC5B genotype (all discrete variables) between the group with ILD changes and the group without ILD changes present on enrollment HRCT
Patient reported cough score
Time frame: Baseline
Difference in visual analogue scale (VAS) for cough (scores between 0-100, with 100 being the worst) between the group with ILD changes and the group without ILD changes present on enrollment HRCT
Patient reported dyspnea score
Time frame: Baseline
Difference in medical research council (MRC) (scores between 0-5, with 5 being the worst) between the group with ILD changes and the group without ILD changes present on enrollment HRCT
Clubbing
Time frame: Baseline
Difference in presence of digital clubbing between the group with ILD changes and the group without ILD changes present on enrollment HRCT
Crackles
Time frame: Baseline
Difference in inspiratory crackles during lung auscultation between the group with ILD changes and the group without ILD changes present on enrollment HRCT
13 secondary outcomes
Forced Vital Capacity (FVC)
Time frame: 2 years
Haemoglobulin-corrected carbon monoxide diffusing capacity (DLCOc)
Time frame: 2 years
Total lung capacity (TLC)
Time frame: 2 years
Biomarker levels
Time frame: 2 years
Genotype
Time frame: 2 years
Oxygen saturation in 6-minute walk test (6MWT)
Time frame: 2 years
Distance (meters) in 6-minute walk test (6MWT)
Time frame: 2 years
Patient reported cough score
Time frame: 2 years
Patient reported dyspnea score
Time frame: 2 years
Digital clubbing
Time frame: 2 years
Crackles
Time frame: 2 years
Patient reported fatigue
Time frame: 2 years
Patient reported health status
Time frame: 2 years
Results and safety
Results are not available in the source record.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 10 May 2022.
- Individual participant dataSharing planned: No. Data of patients is coded
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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.
1 site in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
An observational study of Pulmonary Fibrosis Idiopathic Familial, run by St. Antonius Hospital at 1 site in Netherlands.
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 1 inclusion and 4 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: status not confirmed, as of the sponsor's last update on 10 May 2022. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 1 site in Netherlands. 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?
Results are not available in the source record.
How can a doctor refer a patient or contact the research team?
Contact Jan Grutters, Prof. (+31883201453, j.grutters@antoniusziekenhuis.nl), or a site near the patient. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
Characteristics of participants (time frame: Baseline). The study lists 12 primary outcomes in all. It is what the study was designed to measure, not a finding.
Where can I read the original study information?
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 10 May 2022.