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

The objectives of this study are as follows:

Specific Aim 1: Assemble a network of clinical centers to procure biologic samples from
subjects with recently diagnosed IPF and follow these subjects for at least 48 weeks.
Specific Aim 2: Correlate and integrate biologically plausible biomarkers of disease
activity obtained from multiple compartments (SLB, BAL, TBB, blood) from the same subject
with longitudinal measures of disease progression (change in forced vital capacity, change
in diffusion capacity for carbon monoxide, acute exacerbation of pulmonary fibrosis, and
death).

General Study Design This study will take place in two phases. During the first phase of
the study we will identify and collect baseline specimens from subjects with either
suspected or recently diagnosed (within 48 months) IPF. During the second phase of the study
subjects with IPF will be followed from between 48 and 80 weeks. Subjects will be followed
until the end of study (2 year grant award) or until they meet any part of a composite
endpoint (death, acute exacerbation of IPF, relative decline in FVC of at least 10% or DLCO
of 15%). This is a prospective cohort study. There is no treatment prescribed or studied
as part of this prospective cohort study. Subjects are able to utilize any treatments
prescribed by their physician, including participation in clinical trials as long as they
are able to comply with the follow up schedule in this study.

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