Pulmonary Fibrosis Biomarker Cohort - a Prospective Cohort of Incident Patients With IPF (PFBIO)

Pulmonary Fibrosis Biomarker Cohort (PFBIO)

Incident patients with idiopathic pulmonary fibrosis (IPF) in Denmark will be offered inclusion and followed up for up to 5 years with measurements of blood biomarkers and measurements of disease progression.

Study Overview

• Status: Active, not recruiting
• Conditions: Idiopathic Pulmonary Fibrosis

Detailed Description

IPF pathogenesis is complex, including epithelial injury, resident fibroblast-myofibroblast transformation, recruitment of fibrocytes, macrophage activation, and release of numerous cytokines and chemokines. Several of these processes release potential biomarker proteins into the blood stream or onto the epithelial surface where they can be measured. Biomarkers have mainly two potential roles in IPF. Firstly, a diagnostic biomarker would distinguish IPF from other diseases with similar symptoms, facilitating diagnosis and possibly decreasing the need for risky procedures, such as surgical lung biopsy. Secondly, a prognostic biomarker would distinguish rapid progressors from slow progressors, which is difficult today.

This study will prospectively include patients at the two largest centres in Denmark where patients are treated for IPF and has thus a good opportunity to include the majority of incident cases of IPF in Denmark. The blood levels of several promising biomarkers will be measured at baseline and during up to 5 years follow-up. Patients will also be followed up through regular clinical examination and by querying national registries to determine disease progression, mortality, healthcare utilization and selected co-morbidities. The database will be used for determination of risk factors for the outcomes listed above. Sub-group analyses are planned in respect to sex, treatment, radiologic imaging, smoking status, clinical data such as pulmonary function tests, co-morbidities (both pulmonary disease and extra-pulmonary disease), and disease severity at baseline.

A research biobank with blood samples is established from the study population. This biobank, and the database of newly diagnosed IPF patients, will be used for future research in IPF.

The prospectively created database will also be used for future research in IPF.

• Study Type: Observational
• Enrollment (Estimated): 450

Contacts and Locations

Study Locations

• Denmark
  • Aarhus, Denmark, 8000
    • Aarhus University Hospital
  • Copenhagen
    • Hellerup, Copenhagen, Denmark, 2900
      • Gentofte Hospital

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: No

• Sampling Method: Probability Sample

Study Population

Incidental patients diagnosed with idiopathic pulmonary fibrosis (IPF) at Gentofte hospital and Aarhus university hospital are screened for inclusion

Description

Inclusion Criteria:

• Diagnosis of idiopathic pulmonary fibrosis according to the 2011 guidelines by the American Thoracic Cosicety (ATS) and European Respiratory Society (ERS)

Exclusion Criteria:

• Age lower than 18 years
• Unable to provide informed consent to participation

Study Plan

How is the study designed?

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Disease progression or mortality	Number of patients who fulfil any of the following: disease progression or death	1 year

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Hospitalizations	Number of respiratory and non-respiratory hospitalizations	1 year
Exacerbations	Number of acute exacerbations of idiopathic pulmonary fibrosis	1 year
Lung function tests	Reduction in diffusion capacity (DLCO) and forced vital capacity (FVC)	1 year
Mortality	All-cause and disease-specific mortality	1 year
Change in quality of life	Change in St. George Respiratory Questionnaire, symptom scores	1 year
Combined end-point of disease progression	Number of patients who fulfill any of the following: decrease in lung function, reduced walking distance at 6 minutes walking test, increased need for supplementary oxygen, hospitalization	1 year
Progression in serum/plasma biomarker levels	Increase or decrease in serum/plasma biomarker levels.	1 year

Collaborators and Investigators

• Sponsor: Nils Hoyer
• Collaborators: Aarhus University Hospital; Nordic Bioscience A/S
• Investigators: Principal Investigator: Nils Hoyer, MD, Gentofte Hospital

Publications and helpful links

General Publications

• Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK, Colby TV, Cordier JF, Flaherty KR, Lasky JA, Lynch DA, Ryu JH, Swigris JJ, Wells AU, Ancochea J, Bouros D, Carvalho C, Costabel U, Ebina M, Hansell DM, Johkoh T, Kim DS, King TE Jr, Kondoh Y, Myers J, Muller NL, Nicholson AG, Richeldi L, Selman M, Dudden RF, Griss BS, Protzko SL, Schunemann HJ; ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011 Mar 15;183(6):788-824. doi: 10.1164/rccm.2009-040GL.
• Hoyer N, Prior TS, Bendstrup E, Shaker SB. Diagnostic delay in IPF impacts progression-free survival, quality of life and hospitalisation rates. BMJ Open Respir Res. 2022 Jul;9(1):e001276. doi: 10.1136/bmjresp-2022-001276.

Study record dates

Study Major Dates

• Study Start: April 1, 2016
• Primary Completion (Estimated): December 1, 2023
• Study Completion (Estimated): December 1, 2028

Study Registration Dates

• First Submitted: April 19, 2016
• First Submitted That Met QC Criteria: April 25, 2016
• First Posted (Estimated): April 29, 2016

Study Record Updates

• Last Update Posted (Actual): December 22, 2023
• Last Update Submitted That Met QC Criteria: December 21, 2023
• Last Verified: December 1, 2023

More Information

Terms related to this study

• Keywords: Biomarker; Prognosis; Diagnosis
• Additional Relevant MeSH Terms: Pathologic Processes; Respiratory Tract Diseases; Lung Diseases; Lung Diseases, Interstitial; Fibrosis; Pulmonary Fibrosis; Idiopathic Pulmonary Fibrosis
• Other Study ID Numbers: PFBIO

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: UNDECIDED