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Optimising Screening for Early Disease Detection in Familial Pulmonary Fibrosis (FLORIS)

4 de maio de 2022 atualizado 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.

Visão geral do estudo

Status

Recrutamento

Condições

Descrição detalhada

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 estudo

Observacional

Inscrição (Antecipado)

200

Contactos e Locais

Esta seção fornece os detalhes de contato para aqueles que conduzem o estudo e informações sobre onde este estudo está sendo realizado.

Contato de estudo

Estude backup de contato

Locais de estudo

Critérios de participação

Os pesquisadores procuram pessoas que se encaixem em uma determinada descrição, chamada de critérios de elegibilidade. Alguns exemplos desses critérios são a condição geral de saúde de uma pessoa ou tratamentos anteriores.

Critérios de elegibilidade

Idades elegíveis para estudo

18 anos e mais velhos (Adulto, Adulto mais velho)

Aceita Voluntários Saudáveis

Não

Gêneros Elegíveis para o Estudo

Tudo

Método de amostragem

Amostra Não Probabilística

População do estudo

Asymptomatic first-degree relatives of patients with FPF are invited to enroll.

Descrição

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.

Plano de estudo

Esta seção fornece detalhes do plano de estudo, incluindo como o estudo é projetado e o que o estudo está medindo.

Como o estudo é projetado?

Detalhes do projeto

  • Modelos de observação: Coorte
  • Perspectivas de Tempo: Prospectivo

O que o estudo está medindo?

Medidas de resultados primários

Medida de resultado
Descrição da medida
Prazo
Characteristics of participants
Prazo: 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)
Prazo: 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)
Prazo: 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)
Prazo: 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
Prazo: 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)
Prazo: 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)
Prazo: 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
Prazo: 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
Prazo: 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
Prazo: 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
Prazo: 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
Prazo: 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

Medidas de resultados secundários

Medida de resultado
Descrição da medida
Prazo
Forced Vital Capacity (FVC)
Prazo: 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)
Prazo: 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)
Prazo: 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
Prazo: 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
Prazo: 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)
Prazo: 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)
Prazo: 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
Prazo: 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
Prazo: 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
Prazo: 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
Prazo: 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
Prazo: 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
Prazo: 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

Outras medidas de resultado

Medida de resultado
Descrição da medida
Prazo
Relatedness
Prazo: Baseline
In case multiple members of the same family are included in the study
Baseline
Blood count
Prazo: 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
Prazo: 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
Prazo: 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
Prazo: 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
Prazo: 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

Colaboradores e Investigadores

É aqui que você encontrará pessoas e organizações envolvidas com este estudo.

Patrocinador

St. Antonius Hospital

Colaboradores

ZonMw: The Netherlands Organisation for Health Research and Development
Boeringer Ingelheim

Datas de registro do estudo

Essas datas acompanham o progresso do registro do estudo e os envios de resumo dos resultados para ClinicalTrials.gov. Os registros do estudo e os resultados relatados são revisados ​​pela National Library of Medicine (NLM) para garantir que atendam aos padrões específicos de controle de qualidade antes de serem publicados no site público.

Datas Principais do Estudo

Início do estudo (Real)

16 de junho de 2021

Conclusão Primária (Antecipado)

16 de junho de 2025

Conclusão do estudo (Antecipado)

1 de setembro de 2025

Datas de inscrição no estudo

Enviado pela primeira vez

28 de março de 2022

Enviado pela primeira vez que atendeu aos critérios de CQ

4 de maio de 2022

Primeira postagem (Real)

10 de maio de 2022

Atualizações de registro de estudo

Última Atualização Postada (Real)

10 de maio de 2022

Última atualização enviada que atendeu aos critérios de controle de qualidade

4 de maio de 2022

Última verificação

1 de março de 2022

Mais Informações

Termos relacionados a este estudo

Palavras-chave

Termos MeSH relevantes adicionais

Outros números de identificação do estudo

  • NL75303.100.20

Plano para dados de participantes individuais (IPD)

Planeja compartilhar dados de participantes individuais (IPD)?

Não

Descrição do plano IPD

Data of patients is coded

Informações sobre medicamentos e dispositivos, documentos de estudo

Estuda um medicamento regulamentado pela FDA dos EUA

Não

Estuda um produto de dispositivo regulamentado pela FDA dos EUA

Não

Essas informações foram obtidas diretamente do site clinicaltrials.gov sem nenhuma alteração. Se você tiver alguma solicitação para alterar, remover ou atualizar os detalhes do seu estudo, entre em contato com register@clinicaltrials.gov. Assim que uma alteração for implementada em clinicaltrials.gov, ela também será atualizada automaticamente em nosso site .

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