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.
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Characteristics of participants
Timeframe: Baseline
Forced Vital Capacity (FVC)
Timeframe: Baseline
Haemoglobulin-corrected carbon monoxide diffusing capacity (DLCOc)
Timeframe: Baseline
Total lung capacity (TLC)
Timeframe: Baseline
Biomarker levels
Timeframe: Baseline
Oxygen saturation in 6-minute walk test (6MWT)
Timeframe: Baseline
Distance (meters) in 6-minute walk test (6MWT)
Timeframe: Baseline
MUC5B genotype
Timeframe: Baseline
Patient reported cough score
Timeframe: Baseline
Patient reported dyspnea score
Timeframe: Baseline
Clubbing
Timeframe: Baseline
Crackles
Timeframe: Baseline