Optimising Screening for Early Disease Detection in Familial Pulmonary Fibrosis (FLORIS)
4 de mayo de 2022 actualizado por: 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.
Descripción general del estudio
Estado
Reclutamiento
Condiciones
Descripción detallada
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.
Tipo de estudio
De observación
Inscripción (Anticipado)
200
Contactos y Ubicaciones
Esta sección proporciona los datos de contacto de quienes realizan el estudio e información sobre dónde se lleva a cabo este estudio.
Estudio Contacto
- Nombre: Jan Grutters, Prof.
- Número de teléfono: +31883201453
- Correo electrónico: j.grutters@antoniusziekenhuis.nl
Copia de seguridad de contactos de estudio
- Nombre: Martijn Maus, MD
- Número de teléfono: +31883201467
- Correo electrónico: m.maus@antoniusziekenhuis.nl
Ubicaciones de estudio
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Nieuwegein, Países Bajos, 3435CM
- Reclutamiento
- St Antonius hospital
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Contacto:
- Jan Grutters, Prof.
- Número de teléfono: +31883201453
- Correo electrónico: j.grutters@antoniusziekenhuis.nl
-
Contacto:
- Martijn Maus, MD
- Número de teléfono: +31883201467
- Correo electrónico: m.maus@antoniusziekenhuis.nl
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-
Criterios de participación
Los investigadores buscan personas que se ajusten a una determinada descripción, denominada criterio de elegibilidad. Algunos ejemplos de estos criterios son el estado de salud general de una persona o tratamientos previos.
Criterio de elegibilidad
Edades elegibles para estudiar
18 años y mayores (Adulto, Adulto Mayor)
Acepta Voluntarios Saludables
No
Géneros elegibles para el estudio
Todos
Método de muestreo
Muestra no probabilística
Población de estudio
Asymptomatic first-degree relatives of patients with FPF are invited to enroll.
Descripción
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 de estudios
Esta sección proporciona detalles del plan de estudio, incluido cómo está diseñado el estudio y qué mide el estudio.
¿Cómo está diseñado el estudio?
Detalles de diseño
- Modelos observacionales: Grupo
- Perspectivas temporales: Futuro
¿Qué mide el estudio?
Medidas de resultado primarias
Medida de resultado |
Medida Descripción |
Periodo de tiempo |
|---|---|---|
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Characteristics of participants
Periodo de tiempo: Baseline
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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
|
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Forced Vital Capacity (FVC)
Periodo de tiempo: 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)
Periodo de tiempo: Baseline
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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
|
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Total lung capacity (TLC)
Periodo de tiempo: 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
Periodo de tiempo: Baseline
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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)
Periodo de tiempo: Baseline
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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
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Distance (meters) in 6-minute walk test (6MWT)
Periodo de tiempo: Baseline
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Difference in distance covered (in meters) between the group with ILD changes and the group without ILD changes present on enrollment HRCT
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Baseline
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MUC5B genotype
Periodo de tiempo: Baseline
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Difference in MUC5B genotype (all discrete variables) between the group with ILD changes and the group without ILD changes present on enrollment HRCT
|
Baseline
|
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Patient reported cough score
Periodo de tiempo: Baseline
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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
|
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Patient reported dyspnea score
Periodo de tiempo: Baseline
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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
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Baseline
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Clubbing
Periodo de tiempo: Baseline
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Difference in presence of digital clubbing between the group with ILD changes and the group without ILD changes present on enrollment HRCT
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Baseline
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Crackles
Periodo de tiempo: Baseline
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Difference in inspiratory crackles during lung auscultation between the group with ILD changes and the group without ILD changes present on enrollment HRCT
|
Baseline
|
Medidas de resultado secundarias
Medida de resultado |
Medida Descripción |
Periodo de tiempo |
|---|---|---|
|
Forced Vital Capacity (FVC)
Periodo de tiempo: 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)
Periodo de tiempo: 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
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2 years
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Total lung capacity (TLC)
Periodo de tiempo: 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
|
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Biomarker levels
Periodo de tiempo: 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
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2 years
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Genotype
Periodo de tiempo: 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)
Periodo de tiempo: 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
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2 years
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Distance (meters) in 6-minute walk test (6MWT)
Periodo de tiempo: 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
Periodo de tiempo: 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
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2 years
|
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Patient reported dyspnea score
Periodo de tiempo: 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
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2 years
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Digital clubbing
Periodo de tiempo: 2 years
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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
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2 years
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Crackles
Periodo de tiempo: 2 years
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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
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2 years
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Patient reported fatigue
Periodo de tiempo: 2 years
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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
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2 years
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Patient reported health status
Periodo de tiempo: 2 years
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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
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2 years
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Otras medidas de resultado
Medida de resultado |
Medida Descripción |
Periodo de tiempo |
|---|---|---|
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Relatedness
Periodo de tiempo: Baseline
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In case multiple members of the same family are included in the study
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Baseline
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Blood count
Periodo de tiempo: 2 years
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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
Periodo de tiempo: 2 years
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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
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2 years
|
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Liver function tests
Periodo de tiempo: 2 years
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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
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2 years
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Total bilirubin
Periodo de tiempo: 2 years
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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
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2 years
|
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Additional blood based markers
Periodo de tiempo: 2 years
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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
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2 years
|
Colaboradores e Investigadores
Aquí es donde encontrará personas y organizaciones involucradas en este estudio.
Patrocinador
Fechas de registro del estudio
Estas fechas rastrean el progreso del registro del estudio y los envíos de resultados resumidos a ClinicalTrials.gov. Los registros del estudio y los resultados informados son revisados por la Biblioteca Nacional de Medicina (NLM) para asegurarse de que cumplan con los estándares de control de calidad específicos antes de publicarlos en el sitio web público.
Fechas importantes del estudio
Inicio del estudio (Actual)
16 de junio de 2021
Finalización primaria (Anticipado)
16 de junio de 2025
Finalización del estudio (Anticipado)
1 de septiembre de 2025
Fechas de registro del estudio
Enviado por primera vez
28 de marzo de 2022
Primero enviado que cumplió con los criterios de control de calidad
4 de mayo de 2022
Publicado por primera vez (Actual)
10 de mayo de 2022
Actualizaciones de registros de estudio
Última actualización publicada (Actual)
10 de mayo de 2022
Última actualización enviada que cumplió con los criterios de control de calidad
4 de mayo de 2022
Última verificación
1 de marzo de 2022
Más información
Términos relacionados con este estudio
Palabras clave
Términos MeSH relevantes adicionales
Otros números de identificación del estudio
- NL75303.100.20
Plan de datos de participantes individuales (IPD)
¿Planea compartir datos de participantes individuales (IPD)?
No
Descripción del plan IPD
Data of patients is coded
Información sobre medicamentos y dispositivos, documentos del estudio
Estudia un producto farmacéutico regulado por la FDA de EE. UU.
No
Estudia un producto de dispositivo regulado por la FDA de EE. UU.
No
Esta información se obtuvo directamente del sitio web clinicaltrials.gov sin cambios. Si tiene alguna solicitud para cambiar, eliminar o actualizar los detalles de su estudio, comuníquese con register@clinicaltrials.gov. Tan pronto como se implemente un cambio en clinicaltrials.gov, también se actualizará automáticamente en nuestro sitio web. .