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.

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.

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

Patients with an MDT diagnosis of idiopathic pulmonary fibrosis attending routine follow-up care