Key inclusion and exclusion criteria

Inclusion criteriaExclusion criteria
Adult male or female patients with documented diagnosis of IPF according to the following criteria:
  • Exclusion of other known causes of interstitial lung disease (e.g. domestic and occupational environmental exposures, connective tissue disease and drug toxicity)#

  • AND

    • Combination of patterns of UIP on HRCT images of chest and on surgical lung biopsy (when obtained)#

  • OR

    • Presence of possible UIP pattern# on HRCT images of chest AND additional evidence of traction bronchiectasis as assessed by an experienced chest radiologist (central review)

Signed written informed consent
Age ≤40 years IPF disease diagnosis >5 years FVC % pred <40%DLCO corrected for haemoglobin % pred <30% Severe chronic obstructive bronchitis as characterised by FEV1/FVC <0.70 Known diagnosis of significant respiratory disorders other than IPF Pulmonary artery hypertension requiring a specific treatment Currently listed and/or anticipated to be listed for lung transplantation within the next 6 months (on an active list) History of vasculitis or connective tissue disorders or antineutrophil cytoplasmic auto-antibody positive Acute myocardial infarction within 6 months prior to screening

IPF: idiopathic pulmonary fibrosis; UIP: usual interstitial pneumonia; HRCT: high-resolution computed tomography; FVC: forced vital capacity; DLCO: diffusing capacity of the lung for carbon monoxide; FEV1: forced expiratory volume in 1 s. #: as defined by 2011 American Thoracic Society/European Respiratory Society/Japanese Respiratory Society/Latin American Thoracic Association guidelines [1]; : the patterns of UIP on HRCT images of chest and histopathology in surgical lung biopsy (if conducted) were reviewed by central reviewers (a radiologist and a pathologist experienced in interstitial lung diseases) to confirm diagnosis of IPF for consistency.