Longitudinal Changes in Serum KL-6 in IPF

Study Overview
=================
Brief Summary
-----------------
Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease with a poor prognosis. More accurate tests to predict disease progression and response to treatment are required. Krebs von den Lungen-6 (KL-6) is a blood marker associated with IPF. Results from previous studies have shown that levels of KL-6 are higher in patients with IPF compared to people without the disease. In addition, it is not clear what impact treatment has on KL-6 levels, and whether this could help us to monitor how effective treatment for IPF is. The investigators plan to perform a study in which KL-6 levels in the blood of patients with a new diagnosis of IPF are measured at baseline, 3, 6 and 12 months to look for and changes in the levels of KL-6 in the blood.

Detailed Description
-----------------
Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease with a poor prognosis. Effective treatment which slows the progression of IPF has recently become available however, it is costly and at present is limited to patients who meet specific criteria based on their breathing tests. The breathing tests currently available to monitor progression of the disease are not always reliable and do not predict which patients will respond to treatment. More accurate tests to predict disease progression and response to treatment are required. Krebs von den Lungen-6 (KL-6) is a blood marker associated with IPF. Results from previous studies have shown that levels of KL-6 are higher in patients with IPF compared to people without the disease. The majority of studies using KL-6 in IPF have taken place in Japan and there is limited evidence of how useful it is in a European population. In addition, it is not clear what impact treatment has on KL-6 levels, and whether this could help us to monitor how effective treatment for IPF is. The investigators plan to perform a study in which KL-6 levels in the blood of patients with a new diagnosis of IPF are measured at baseline, 3, 6 and 12 months to look for and changes in the levels of KL-6 in the blood The objective of this study is to assess changes in serum KL-6 levels in patients with IPF over a 12-month period and assess if this correlates with changes in lung function and if KL-6 levels change in response to treatment with antifibrotic therapy.

Official Title
-----------------
Longitudinal Changes in Serum KL-6 Levels in Idiopathic Pulmonary Fibrosis (LOCK-IPF)

Conditions
-----------------
Idiopathic Pulmonary Fibrosis

Intervention / Treatment
-----------------
* Diagnostic Test: Serum KL-6 level


Participation Criteria
=================
Eligibility Criteria
-----------------
Inclusion Criteria: Age 18 or older Multi-disciplinary team (MDT) diagnosis of idiopathic pulmonary fibrosis as per international consensus guidelines Exclusion criteria Significant respiratory co-morbidity (i.e. where the major respiratory diagnosis is not IPF) FEV1/FVC ratio < 70% on full lung function testing Current smoker (within 4 week of enrollment) Received treatment for acute lower respiratory tract infection with last 4 weeks Use of long-term (greater than 4 weeks) oral corticosteroids or immunosuppression within 4 weeks of enrolment Current participation in a double-blind placebo-controlled pharmaceutical trial

Ages Eligible for Study
-----------------
Minimum Age: 18 Years

Sexes Eligible for Study
-----------------
All

Accepts Healthy Volunteers
-----------------
No

Study Plan
=================
How is the study designed?
-----------------

Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| IPF patients<br>Patients with an MDT diagnosis of idiopathic pulmonary fibrosis. Patients will be observed over a 12 month period and have serial serum samples taken for KL-6 level. | Diagnostic Test: Serum KL-6 level<br>* Serum blood biomarker which has been shown to be of interest in idiopathic pulmonary fibrosis<br>|

What is the study measuring?
-----------------
Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Serum KL-6 level | Change in serum KL-6 level between baseline and 12 months | 12 months | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Serum KL-6 level at 3, 6 months | Change in serum KL-6 at 3 and 6 months compared to baseline | 3 and 6 months | 
| KL-6 forced vital capacity (FVC) correlation | Correlation of KL-6 and FVC change at 3, 6 and 12 months | 3, 6 and12 months | 
| KL-6 diffusion capacity (DLCO) | Correlation of KL-6 and DLCO change at 3, 6 and 12 months | 3, 6 and12 months | 
| KL-6 symptoms | Correlation of KL-6 and symptom scores at 3, 6 and 12 months | 3, 6 and12 months | 
| KL-6 antifibrotics | Change in KL-6 levels in response to antifibrotic therapy | 12 months | 
| KL-6 Gender Age and Physiology (GAP) stage | Differences in KL-6 levels between Gender Age Physiology (GAP) stage at baseline | At baseline | 
| KL-6 CPI | Correlation between KL-6 levels and Composite Physiology Index (CPI) | At baseline | 
| KL-6 CT pattern | Difference in KL-6 levels between patients with indeterminate, probable and definite usual interstitial pneumonia pattern (UIP) on HRCT | At baseline | 

 Terms related to the study
=================
Keywords Provided by Centre Hospitalier Valida
-----------------
KL-6, Krebs von den Lungen-6, Biomarker