Idiopathic Pulmonary Fibrosis
Conditions
Keywords
KL-6, Krebs von den Lungen-6, Biomarker
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.
Interventions
DIAGNOSTIC_TESTSerum KL-6 level
Serum blood biomarker which has been shown to be of interest in idiopathic pulmonary fibrosis
Sponsors
Manchester University NHS Foundation Trust
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Serum KL-6 level | 12 months | Change in serum KL-6 level between baseline and 12 months |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| KL-6 forced vital capacity (FVC) correlation | 3, 6 and12 months | Correlation of KL-6 and FVC change at 3, 6 and 12 months |
| KL-6 diffusion capacity (DLCO) | 3, 6 and12 months | Correlation of KL-6 and DLCO change at 3, 6 and 12 months |
| KL-6 symptoms | 3, 6 and12 months | Correlation of KL-6 and symptom scores at 3, 6 and 12 months |
| Serum KL-6 level at 3, 6 months | 3 and 6 months | Change in serum KL-6 at 3 and 6 months compared to baseline |
| KL-6 Gender Age and Physiology (GAP) stage | At baseline | Differences in KL-6 levels between Gender Age Physiology (GAP) stage at baseline |
| KL-6 CPI | At baseline | Correlation between KL-6 levels and Composite Physiology Index (CPI) |
| KL-6 CT pattern | At baseline | Difference in KL-6 levels between patients with indeterminate, probable and definite usual interstitial pneumonia pattern (UIP) on HRCT |
| KL-6 antifibrotics | 12 months | Change in KL-6 levels in response to antifibrotic therapy |
Countries
United Kingdom
Contacts
Primary ContactConal Hayton, MBChB
Backup ContactNazia Chaudhuri
Outcome results
None listed