Safety and Efficacy of BIBF 1120 at High Dose in Idiopathic Pulmonary Fibrosis Patients

Inclusion criteria:

1. Age >= 40 years;

2. IPF diagnosed, according to most recent American Thoracic Society (ATS), EuropeanRespiratory Society (ERS), Japanese Respiratory Society (JRS), Latin American ThoracicAssociation (ALAT) IPF guideline for diagnosis and management, within 5 years;

3. Combination of High Resolution Computerized Tomography (HRCT) pattern, and ifavailable surgical lung biopsy pattern, as assessed by central reviewers, areconsistent with diagnosis of IPF

4. Dlco (corrected for Hb): 30%-79% predicted of normal;

5. FVC>= 50% predicted of normal

Exclusion criteria:

1. Aspartate Aminotransferase (AST), Alanine Aminotransferase (ALT) > 1.5 x Upper Limitof Normal (ULN)

2. Bilirubin > 1.5 x ULN;

3. Relevant airways obstruction (i.e. pre-bronchodilator FEV1/FVC < 0.7);

4. Patient likely to have lung transplantation during study (being on transplantationlist is acceptable for participation);

5. Myocardial infarction within 6 months;

6. Unstable angina within 1 month;

7. Bleeding risk (genetic predisposition; fibrinolysis or full-dose therapeuticanticoagulation or high dose antiplatelet therapy; history of hemorrhagic CNS eventwithin 12 months; haemoptysis or haematuria or active gastro-intestinal bleeding orulcers or major injury or surgery within 3 months);

8. Thrombotic risk (inherited predisposition; history of thrombotic event (includingstroke and transient ischemic attacks) within 12 months;

9. International normalised ratio (INR) > 2, prolongation of prothrombin time (PT) andpartial thromboplastin time (PTT) by > 50% of institutional ULN);

10. N-ACetyl Cystein, prednisone > 15mg/day or equivalent received within 2 weeks of visit 1;

11. Pirfenidone, azathioprine, cyclophosphamide, cyclosporine A received within 8 weeks ofvisit 1;