Correlating Outcomes With Biochemical Markers to Estimate Time-progression in Idiopathic Pulmonary Fibrosis (IPF)

Inclusion Criteria: 1. Suspected or confirmed diagnosis of IPF 2. Age 35 - 80 years inclusive 3. Ability to understand and provide informed consent Exclusion Criteria: 1. Confirmed diagnosis of IPF at the study center more than 4 years prior to screening 2. Environmental exposure (occupational, environmental, drug, etc) felt by the principal investigator (PI) to be the etiology of the interstitial disease 3. Diagnosis of collagen-vascular conditions (according to the published American College of Rheumatology criteria) 4. Forced expiratory volume in 1 second (FEV1)/FVC ratio \< 0.60 at screening (postbronchodilator) 5. Significant bronchodilator response on screening spirometry, defined as a change in FEV1 ≥ 12% and absolute change \> 200 mL OR change in FVC ≥ 12% and absolute change \> 200 mL 6. Evidence of active infection at screening 7. Listed for lung transplantation at time of screening 8. Unstable or deteriorating cardiac disease at screening 9. Myocardial infarction, coronary artery bypass, or angioplasty within 6 months of screening 10. Unstable angina pectoris or congestive heart failure requiring hospitalization within 6 months of screening 11. Uncontrolled arrhythmia at screening 12. Severe uncontrolled hypertension at screening 13. Known HIV or hepatitis C at screening 14. Known cirrhosis or chronic active hepatitis at screening 15. Active substance and/or alcohol abuse at screening 16. Subjects who are pregnant or breastfeeding at screening 17. Women of childbearing potential who are not using a medically approved means of contraception at screening 18. Known bleeding abnormality that would preclude the performance of transbronchial lung biopsy 19. Prothrombin time, INR \> 1.5, Partial Thromboplastin Time (PTT) \> 45 at time of screening, platelets \< 100,000/mm3 20. Any condition other than IPF that, in the opinion of the site PI, is likely to result in the death of the subject within the next year 21. Any condition that, in the judgment of the site PI, might cause participation in this study to be detrimental to the subject or that the site PI deems makes the subject a poor candidate