Optimising Screening for Early Disease Detection in Familial Pulmonary Fibrosis (FLORIS)
4 mai 2022 mis à jour par: 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.
Aperçu de l'étude
Statut
Recrutement
Les conditions
Description détaillée
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.
Type d'étude
Observationnel
Inscription (Anticipé)
200
Contacts et emplacements
Cette section fournit les coordonnées de ceux qui mènent l'étude et des informations sur le lieu où cette étude est menée.
Coordonnées de l'étude
- Nom: Jan Grutters, Prof.
- Numéro de téléphone: +31883201453
- E-mail: j.grutters@antoniusziekenhuis.nl
Sauvegarde des contacts de l'étude
- Nom: Martijn Maus, MD
- Numéro de téléphone: +31883201467
- E-mail: m.maus@antoniusziekenhuis.nl
Lieux d'étude
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Nieuwegein, Pays-Bas, 3435CM
- Recrutement
- St Antonius hospital
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Contact:
- Jan Grutters, Prof.
- Numéro de téléphone: +31883201453
- E-mail: j.grutters@antoniusziekenhuis.nl
-
Contact:
- Martijn Maus, MD
- Numéro de téléphone: +31883201467
- E-mail: m.maus@antoniusziekenhuis.nl
-
-
Critères de participation
Les chercheurs recherchent des personnes qui correspondent à une certaine description, appelée critères d'éligibilité. Certains exemples de ces critères sont l'état de santé général d'une personne ou des traitements antérieurs.
Critère d'éligibilité
Âges éligibles pour étudier
18 ans et plus (Adulte, Adulte plus âgé)
Accepte les volontaires sains
Non
Sexes éligibles pour l'étude
Tout
Méthode d'échantillonnage
Échantillon non probabiliste
Population étudiée
Asymptomatic first-degree relatives of patients with FPF are invited to enroll.
La description
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.
Plan d'étude
Cette section fournit des détails sur le plan d'étude, y compris la façon dont l'étude est conçue et ce que l'étude mesure.
Comment l'étude est-elle conçue ?
Détails de conception
- Modèles d'observation: Cohorte
- Perspectives temporelles: Éventuel
Que mesure l'étude ?
Principaux critères de jugement
Mesure des résultats |
Description de la mesure |
Délai |
|---|---|---|
|
Characteristics of participants
Délai: 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)
Délai: 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)
Délai: 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)
Délai: 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
Délai: 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)
Délai: 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)
Délai: 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
Délai: 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
Délai: 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
Délai: 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
Délai: 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
Délai: 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
|
Mesures de résultats secondaires
Mesure des résultats |
Description de la mesure |
Délai |
|---|---|---|
|
Forced Vital Capacity (FVC)
Délai: 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)
Délai: 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)
Délai: 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
Délai: 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
Délai: 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)
Délai: 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)
Délai: 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
Délai: 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
Délai: 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
Délai: 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
Délai: 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
Délai: 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
Délai: 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
|
Autres mesures de résultats
Mesure des résultats |
Description de la mesure |
Délai |
|---|---|---|
|
Relatedness
Délai: Baseline
|
In case multiple members of the same family are included in the study
|
Baseline
|
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Blood count
Délai: 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
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2 years
|
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Creatinin
Délai: 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
Délai: 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
Délai: 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
Délai: 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
|
Collaborateurs et enquêteurs
C'est ici que vous trouverez les personnes et les organisations impliquées dans cette étude.
Dates d'enregistrement des études
Ces dates suivent la progression des dossiers d'étude et des soumissions de résultats sommaires à ClinicalTrials.gov. Les dossiers d'étude et les résultats rapportés sont examinés par la Bibliothèque nationale de médecine (NLM) pour s'assurer qu'ils répondent à des normes de contrôle de qualité spécifiques avant d'être publiés sur le site Web public.
Dates principales de l'étude
Début de l'étude (Réel)
16 juin 2021
Achèvement primaire (Anticipé)
16 juin 2025
Achèvement de l'étude (Anticipé)
1 septembre 2025
Dates d'inscription aux études
Première soumission
28 mars 2022
Première soumission répondant aux critères de contrôle qualité
4 mai 2022
Première publication (Réel)
10 mai 2022
Mises à jour des dossiers d'étude
Dernière mise à jour publiée (Réel)
10 mai 2022
Dernière mise à jour soumise répondant aux critères de contrôle qualité
4 mai 2022
Dernière vérification
1 mars 2022
Plus d'information
Termes liés à cette étude
Termes MeSH pertinents supplémentaires
Autres numéros d'identification d'étude
- NL75303.100.20
Plan pour les données individuelles des participants (IPD)
Prévoyez-vous de partager les données individuelles des participants (DPI) ?
Non
Description du régime IPD
Data of patients is coded
Informations sur les médicaments et les dispositifs, documents d'étude
Étudie un produit pharmaceutique réglementé par la FDA américaine
Non
Étudie un produit d'appareil réglementé par la FDA américaine
Non
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