Evaluate the Safety and Efficacy of FG-3019 in Patients With Idiopathic Pulmonary Fibrosis

The study has been amended accordingly in February 2016 to further allow for the enrollment
of a subgroup of subjects (N=60) who will be allowed to receive treatment with approved IPF
therapy with pirfenidone or with nintedanib as background therapy.

These additional subjects will be stratified by background therapy, randomized to FG-3019 or
placebo and followed up for 24 weeks. The main objective of the study remains safety. PK
samples to assess drug concentrations will also be collected.

(This substudy portion only applies to US and Canadian centers.)

Enrollment for the main study was completed on 29Jun2016 Enrollment for the sub-study was
completed on 16Dec2016

Inclusion Criteria:

1. Age 40 to 80 years, inclusive.

2. Diagnosis of IPF as defined by current international guidelines (Raghu, 2011). Each
subject must have one of the following: (1) Usual Interstitial Pneumonia (UIP) Pattern
on an available HRCT scan; or (2) Possible UIP Pattern on an available HRCT scan and
surgical lung biopsy within 4 years of Screening showing UIP Pattern.

3. History of IPF of ≤5 years duration with onset defined as the date of the first
diagnosis of IPF by HRCT or surgical lung biopsy.

4. Interstitial pulmonary fibrosis defined by HRCT scan at Screening, with evidence of
≥10% to <50% parenchymal fibrosis (reticulation) and <25% honeycombing, within the
whole lung, as determined by the HRCT central reader.

5. FVC percent of predicted value ≥55% at Screening.

6. Female subjects of childbearing potential (including those <1 year postmenopausal)
must be willing to use a medically acceptable method of contraception, for example, an
oral contraceptive, depot progesterone, or intrauterine device. Male subjects with
female partners of childbearing potential who are not using birth control as described
above must use a barrier method of contraception (e.g., condom) if not surgically
sterile (i.e., vasectomy).

7. All subjects in Arm A whose FVC percent predicted value is greater than the original
pre-treatment baseline in the Randomized Treatment Period will be offered
participation in an Extended Treatment Period

8. All subjects assigned to Placebo (Arm B) will be offered participation in the Extended
Treatment Phase.

9. For Substudy only: Receiving treatment for IPF with a stable dose of pirfenidone or
with a stable dose of nintedanib for at least 3 months before screening initiation and
willing to continue treatment with pirfenidone or with nintedanib according to the
corresponding approved label and the prescribing physician, including all listed
safety requirements (e.g., liver function tests, avoidance of sunlight and sunlamp
exposure and wearing of sunscreen and protective clothing daily for pirfenidone, and
smoking cessation).

Exclusion Criteria:

1. Women who are pregnant or nursing.

2. Infiltrative lung disease other than IPF, including any of the other types of
idiopathic interstitial pneumonias (Travis, 2013); lung diseases related to exposure
to fibrogenic agents or other environmental toxins or drugs; other types of
occupational lung diseases; granulomatous lung diseases; pulmonary vascular diseases;
systemic diseases, including vasculitis and connective tissue diseases.

3. HRCT scan findings at Screening are inconsistent with UIP Pattern, as determined by
the HRCT central reader.

4. Pathology diagnosis on surgical lung biopsy is anything other than UIP Pattern, as
determined by the local pathologist.

5. The Investigator judges that there has been sustained improvement in the severity of
IPF during the 12 months prior to Screening, based on changes in FVC, diffusing
capacity of the lung for carbon monoxide (DLCO), and/or HRCT scans of the chest.

6. Clinically important abnormal laboratory tests.

7. Upper or lower respiratory tract infection of any type within 4 weeks of the first
screening visit.

8. Acute exacerbation of IPF within 3 months of the first screening visit.

9. Use of medications to treat IPF within 5 half-lives of Day 1 dosing. If monoclonal
antibodies were used, the last dose of the antibody must be at least 4 weeks before
Day 1 dosing. This applies to subject enrolled in Main Study only.

10. Use of any investigational drugs, including any investigational drugs for IPF, within
4 weeks prior to Day 1 dosing.

11. History of cancer diagnosis of any type in the 3 years preceding Screening, excluding
non-melanomatous skin cancer, localized bladder cancer, or in situ cancers

12. Diffusing capacity (DLCO) less than 30% of predicted value

13. History of allergic or anaphylactic reaction to human, humanized, chimeric or murine
monoclonal antibodies.

14. Previous treatment with FG-3019.

15. Body weight greater than 130 kilograms.