Prospective clinical pilot study for subjects diagnosed with Occupational Progressive Pneumoconiosis.

Subjects will be treated with Nintedanib 150mg twice daily for 3 years.

100 Patients with asbestosis, silicosis, coal workers pneumoconiosis and diffuse dust fibrosis will be included. Patients will have an FVC ≥45% predicted (no upper threshold), and a diffusion capacity of the lung for carbon monoxide (TLCO) above 30% predicted. Patients will be randomised to receive Nintedanib 150 mg twice daily, with the dose of the study drug reduced to 100 mg twice daily or interrupted temporarily in the case of adverse events (AEs).The primary end point will be the annual decline in FVC, measured in millilitres per year, calculated from serial measurements over 36 months.

Lung function testing will be performed at baseline; 2, 4, 6, 12, 18, 24, 30, 36, 44 and 52 weeks, and every 4 months thereafter until study cessation or withdrawal at a maximum of 36 months.

In patients who show clinical benefit as per the end points specified access to Nintedanib treatment will be continued.

Inclusion Criteria:

• Pneumoconiosis diagnosis confirmed at the Occupational MDT (Occ-MDT)
• diffuse fibrosing lung disease of extent >10% on HRCT with protocol criteria for progression
• Asbestosis, silicosis, coal worker's pneumoconiosis and diffuse dust fibrosis
• FVC ≥45% predicted and TLCO above 30% predicted

Exclusion Criteria:

• idiopathic pulmonary fibrosis (IPF) and non-occupational progressive pulmonary fibrosis
• ILD due to connective tissues disorders, hypersensitivity pneumonitis, non-occupational interstitial pneumonia, non-occupational sarcoidosis
• contraindications to Nintedanib (forthcoming surgery, use of anticoagulants, high CVD risk, liver function abnormalities)