Cohort of IPF Patients Experiencing an Exacerbation

Study Overview
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Brief Summary
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Extension of the PFBIO cohort which includes patients with newly diagnosed idiopathic pulmonary fibrosis (IPF) for longitudinal follow-up for up to 5 years. In the PFBIO-EXA extension, patients are included if they experience an exacerbation, or other increase in respiratory symptoms requiring hospital admission, for further collection of clinical and biological data.

Detailed Description
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The aim of this study is to investigate the diagnostic and prognostic value of clinical and blood biomarkers during exacerbations, or other increase in respiratory symptoms requiring hospital admission in patients with IPF. Patients that are already included in the PFBIO-cohort (NCT02755441) and who are admitted to hospital with an increase in respiratory symptoms, are also included in PFBIO-EXA. Patients are recruited within 24 hrs. from hospital admission with respiratory worsening, where clinical data, and blood samples are collected. The blood samples are investigated for the same blood biomarkers as PFBIO. Patient related outcomes are also collected, including quality-of-life questionnaires and outcomes of the exacerbations.

Official Title
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Pulmonary Fibrosis Biomarkers During Exacerbation

Conditions
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Idiopathic Pulmonary Fibrosis

Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Admitted with an increase in respiratory symptoms Already included in PFBIO or included simultaneously in PFBIO and PFBIO-EXA Age at least 18 years Exclusion Criteria: Unable to provide informed consent

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| PFBIO-EXA<br>All patients recruited for PFBIO-EXA from the original PFBIO cohort are included into the cohort. | |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Mortality | All-cause mortality | 30 days | 
| Mortality | All-cause mortality | 90 days | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Biomarker levels | Blood biomarker level | 30 days | 
| LTOT | Change in oxygen need | 30 days | 
| QoL | Score of patient reported quality of life assessed by questionnaires | 30 days | 
| Respiratory support | Need for non-invasive or invasive respiratory support, including oxygen, CPAP, NIV and intubation | 30 days | 
| Decline in functional level | Decrease in functional level, e.g. moved to nursing home, hospice or other | 30 days | 
| Treatment during hospitalization | Treatment administered for respiratory worsening | 30 days | 
| Treatment after hospitalization | Treatment administered after discharge | 90 days | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Exacerbation, Hospitalization, Biomarkers, Outcome, Prognosis, Diagnosis