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

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.

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.

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