Short summary

Interstitial lung diseases (ILD) is a heterogeneous disease category with an annual incidence of approximate 2,000 patients in Denmark. The average survival for patients with fibrotic ILD is 2-5 years contrasting 2 years being the average time to obtain a diagnosis. Therefore, early identification of these patients is of outmost importance. Different ultrasound modalities used to investigate the lungs have shown to be potential 'biomarkers' for both diagnostics and monitoring disease severity.

Rationale

Interstitial lung diseases (ILD) is a heterogeneous disease category of chronic lung diseases with an annual incidence of approximate 2,000 patients in Denmark. Idiopathic pulmonary fibrosis (IPF) is the prototype of so-called progressive fibrosing interstitial lung diseases (PF-ILD). However, nearly 30% of all patients with ILD develop into non-IPF PF-ILD phenotypes with rapid scarring of the lungs (i.e. fibrosis). The average survival for IPF and non-IPF ILD is 2-5 years, which contrasts 2 years being the average time to obtain a diagnosis. Therefore, early identification of these patients is of outmost importance. Different ultrasound modalities used to investigate the lungs, pleura, diaphragm muscle, and pectoralis- and femoral musculature have shown to be potential 'biomarkers' for both diagnostics and monitoring disease severity. However, knowledge on the use of ultrasound in IPF and non-IPF PF-ILD patients is inconsistent investigated. In patients with IPF and non-IPF PF-ILD this PhD project aims to:

1.Examine the existing evidence on the use of ultrasound in patients.

2.Investigate if thoracic ultrasound can be used to assess and monitor the development of disease severity.

3.Investigate whether other ultrasound modalities can be used to assess and monitor development of disease severity.

Description of the cohort

DESIGN AND SETTING Prospective cohort studies of patients with IPF and non-IPF PF-ILD will be recruited from South Danish Center for ILD (SCILS), which acts as a tertiary ILD specialist unit for the Region of Southern Denmark.

MATERIAL AND STUDY PLAN Inclusion criteria: • Age > 18 years • HRCT > 10% fibrosis • Patients with IPF or non-IPF PF-ILD fulfilling at least one of following criteria within a period of 24 months prior to inclusion date : 1. Relative decline in FVC >= 10% pred. 2. Relative decline in FVC >= 5-<10% pred. + worsened respiratory symptoms 3. Relative decline in FVC >= 5-<10% pred. + increased extent of fibrosis on HRCT 4. Worsened respiratory symptoms + increased extent of fibrosis on HRCT • Informed consent Exclusion criteria: • Patients not willing to or able to give informed consent

Data and biological material

DATA COLLECTION Follow-up assessments will be done corresponding to inclusion (= baseline) and after 6 and 12 months. The HRCT must be performed in a window corresponding to 3 months prior to and up to 1 month after inclusion date, and a follow-up HRCT will be performed 6 and 12 months after inclusion date. TUS/US will be conducted on the inclusion date and after 6 and 12 months. Pulmonary function tests including 6-minute walking test (6MWT) and validated patient related outcome measures (PROMs) for health-related quality of life will be performed corresponding to inclusion date, and after 6 and 12 months. Furthermore, after written and oral consent the basic data will be retrieved from the patient journal. All patient information will be pseudo-anonymized and stored in a Research Electronic Data Capture database (REDCap) administrated by Odense Patient data Explorative Network (OPEN).

Collaborating researchers and departments

1. Jesper Rømhild Davidsen

South Danish Center for Interstitial Lung Diseases (SCILS) and Pulmo-RhEuma Clinic (PURE) Department of Respiratory Medicine Odense University Hospital, Odense Denmark

2.Stefan M. Harders, MD PhD

• Department of Radiology Odense University Hospital, Odense Denmark

3.Elisabeth Bendstrup, MD PhD Professor

• Center for Rare Lung Diseases Department of Respiratory Diseases and Allergy Aarhus University Hospital, Aarhus Denmark

4.Michael Thomas Durheim, MD PhD Associate Professor

• Institute of Clinical Medicine Department of Respiratory Medicine Oslo University Hospital Norway

5.Christian B. Laursen, MD PhD Professor

• Department of Respiratory Medicine Odense University Hospital, Odense Denmark

6.Karen Andersen-Ranberg, MD PhD Professor

• Department of Geriatrics Odense University Hospital, Odense Denmark

Publications associated with the project

Awaiting.