Optimising Screening for Early Disease Detection in Familial Pulmonary Fibrosis (FLORIS)
4 maj 2022 uppdaterad av: Jan C. Grutters, MD, St. Antonius Hospital
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.
Studieöversikt
Status
Rekrytering
Betingelser
Detaljerad beskrivning
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.
Studietyp
Observationell
Inskrivning (Förväntat)
200
Kontakter och platser
Det här avsnittet innehåller kontaktuppgifter för dem som genomför studien och information om var denna studie genomförs.
Studiekontakt
- Namn: Jan Grutters, Prof.
- Telefonnummer: +31883201453
- E-post: j.grutters@antoniusziekenhuis.nl
Studera Kontakt Backup
- Namn: Martijn Maus, MD
- Telefonnummer: +31883201467
- E-post: m.maus@antoniusziekenhuis.nl
Studieorter
-
-
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Nieuwegein, Nederländerna, 3435CM
- Rekrytering
- St Antonius hospital
-
Kontakt:
- Jan Grutters, Prof.
- Telefonnummer: +31883201453
- E-post: j.grutters@antoniusziekenhuis.nl
-
Kontakt:
- Martijn Maus, MD
- Telefonnummer: +31883201467
- E-post: m.maus@antoniusziekenhuis.nl
-
-
Deltagandekriterier
Forskare letar efter personer som passar en viss beskrivning, så kallade behörighetskriterier. Några exempel på dessa kriterier är en persons allmänna hälsotillstånd eller tidigare behandlingar.
Urvalskriterier
Åldrar som är berättigade till studier
18 år och äldre (Vuxen, Äldre vuxen)
Tar emot friska volontärer
Nej
Kön som är behöriga för studier
Allt
Testmetod
Icke-sannolikhetsprov
Studera befolkning
Asymptomatic first-degree relatives of patients with FPF are invited to enroll.
Beskrivning
Inclusion Criteria:
- Asymptomatic first-degree relative of patients with familial pulmonary fibrosis (FPF)
Exclusion Criteria:
- 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.
Studieplan
Det här avsnittet ger detaljer om studieplanen, inklusive hur studien är utformad och vad studien mäter.
Hur är studien utformad?
Designdetaljer
- Observationsmodeller: Kohort
- Tidsperspektiv: Blivande
Vad mäter studien?
Primära resultatmått
Resultatmått |
Åtgärdsbeskrivning |
Tidsram |
|---|---|---|
|
Characteristics of participants
Tidsram: 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
|
Baseline
|
|
Forced Vital Capacity (FVC)
Tidsram: Baseline
|
Difference in forced vital capacity (FVC) between the group with ILD changes and the group without ILD changes present on enrollment HRCT
|
Baseline
|
|
Haemoglobulin-corrected carbon monoxide diffusing capacity (DLCOc)
Tidsram: 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
|
Baseline
|
|
Total lung capacity (TLC)
Tidsram: Baseline
|
Difference in total lung capacity (TLC) between the group with ILD changes and the group without ILD changes present on enrollment HRCT
|
Baseline
|
|
Biomarker levels
Tidsram: 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
|
Baseline
|
|
Oxygen saturation in 6-minute walk test (6MWT)
Tidsram: 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
|
Baseline
|
|
Distance (meters) in 6-minute walk test (6MWT)
Tidsram: Baseline
|
Difference in distance covered (in meters) between the group with ILD changes and the group without ILD changes present on enrollment HRCT
|
Baseline
|
|
MUC5B genotype
Tidsram: Baseline
|
Difference in MUC5B genotype (all discrete variables) between the group with ILD changes and the group without ILD changes present on enrollment HRCT
|
Baseline
|
|
Patient reported cough score
Tidsram: 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
|
Baseline
|
|
Patient reported dyspnea score
Tidsram: 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
|
Baseline
|
|
Clubbing
Tidsram: Baseline
|
Difference in presence of digital clubbing between the group with ILD changes and the group without ILD changes present on enrollment HRCT
|
Baseline
|
|
Crackles
Tidsram: Baseline
|
Difference in inspiratory crackles during lung auscultation between the group with ILD changes and the group without ILD changes present on enrollment HRCT
|
Baseline
|
Sekundära resultatmått
Resultatmått |
Åtgärdsbeskrivning |
Tidsram |
|---|---|---|
|
Forced Vital Capacity (FVC)
Tidsram: 2 years
|
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
|
2 years
|
|
Haemoglobulin-corrected carbon monoxide diffusing capacity (DLCOc)
Tidsram: 2 years
|
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
|
2 years
|
|
Total lung capacity (TLC)
Tidsram: 2 years
|
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
|
2 years
|
|
Biomarker levels
Tidsram: 2 years
|
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
|
2 years
|
|
Genotype
Tidsram: 2 years
|
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
|
2 years
|
|
Oxygen saturation in 6-minute walk test (6MWT)
Tidsram: 2 years
|
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
|
2 years
|
|
Distance (meters) in 6-minute walk test (6MWT)
Tidsram: 2 years
|
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
|
2 years
|
|
Patient reported cough score
Tidsram: 2 years
|
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
|
2 years
|
|
Patient reported dyspnea score
Tidsram: 2 years
|
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
|
2 years
|
|
Digital clubbing
Tidsram: 2 years
|
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
|
2 years
|
|
Crackles
Tidsram: 2 years
|
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
|
2 years
|
|
Patient reported fatigue
Tidsram: 2 years
|
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
|
2 years
|
|
Patient reported health status
Tidsram: 2 years
|
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
|
2 years
|
Andra resultatmått
Resultatmått |
Åtgärdsbeskrivning |
Tidsram |
|---|---|---|
|
Relatedness
Tidsram: Baseline
|
In case multiple members of the same family are included in the study
|
Baseline
|
|
Blood count
Tidsram: 2 years
|
Blood count (Consisting of; hemoglobin (mmol/l), erythrocytes (x10^12/l), mean corpuscular volume (fl), red cell distribution width (%), thrombocytes (x10^9/l), leukocytes (x10^9/l)) as an exploratory measurement in the subjects with presence of ILD changes on enrollment HRCT and the difference between bloodcount in the subjects with presence of ILD changes on enrollment HRCT and the group without ILD changes present on enrollment HRCT
|
2 years
|
|
Creatinin
Tidsram: 2 years
|
creatinin (umol/l) as an exploratory measurement in the subjects with presence of ILD changes on enrollment HRCT and the difference between creatinin in the subjects with presence of ILD changes on enrollment HRCT and the group without ILD changes present on enrollment HRCT
|
2 years
|
|
Liver function tests
Tidsram: 2 years
|
Liver function tests (consisting of: ASAT (U/l), ALAT (U/l), alkaline phosphatase (U/l), Gamma-glutamyl transpeptidase (U/l)) as an exploratory measurement in the subjects with presence of ILD changes on enrollment HRCT and the difference between these liver function tests in the subjects with presence of ILD changes on enrollment HRCT and the group without ILD changes present on enrollment HRCT
|
2 years
|
|
Total bilirubin
Tidsram: 2 years
|
Total bilirubin (umol/l) as an exploratory measurement in the subjects with presence of ILD changes on enrollment HRCT and the difference between total bilirubin in the subjects with presence of ILD changes on enrollment HRCT and the group without ILD changes present on enrollment HRCT
|
2 years
|
|
Additional blood based markers
Tidsram: 2 years
|
Additional blood based markers as an exploratory measurement in the subjects with presence of ILD changes on enrollment HRCT and the difference between these blood based markers in the subjects with presence of ILD changes on enrollment HRCT and the group without ILD changes present on enrollment HRCT
|
2 years
|
Samarbetspartners och utredare
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Sponsor
Studieavstämningsdatum
Dessa datum spårar framstegen för inlämningar av studieposter och sammanfattande resultat till ClinicalTrials.gov. Studieposter och rapporterade resultat granskas av National Library of Medicine (NLM) för att säkerställa att de uppfyller specifika kvalitetskontrollstandarder innan de publiceras på den offentliga webbplatsen.
Studera stora datum
Studiestart (Faktisk)
16 juni 2021
Primärt slutförande (Förväntat)
16 juni 2025
Avslutad studie (Förväntat)
1 september 2025
Studieregistreringsdatum
Först inskickad
28 mars 2022
Först inskickad som uppfyllde QC-kriterierna
4 maj 2022
Första postat (Faktisk)
10 maj 2022
Uppdateringar av studier
Senaste uppdatering publicerad (Faktisk)
10 maj 2022
Senaste inskickade uppdateringen som uppfyllde QC-kriterierna
4 maj 2022
Senast verifierad
1 mars 2022
Mer information
Termer relaterade till denna studie
Ytterligare relevanta MeSH-villkor
Andra studie-ID-nummer
- NL75303.100.20
Plan för individuella deltagardata (IPD)
Planerar du att dela individuella deltagardata (IPD)?
Nej
IPD-planbeskrivning
Data of patients is coded
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