Safety and Tolerability Study in Subjects With Idiopathic Pulmonary Fibrosis (IPF)

Inclusion Criteria:

- Male or female subjects ages 21 to 80, inclusive

- Presence of IPF confirmed per ATS criteria (2011)

- Presence of moderate to severe disease, stage II-III defined by GAP index (Gender, Age
and Physiology)

- Subjects who are currently treated with OFEV™/Nintedanib should be on a stable dose
for at least 3 months prior to initiation of the study drug.

- Females of child-bearing potential must have a negative serum ß-hCG (human chorionic
gonadotropin) at screening and must be willing to use appropriate contraception (as
defined by the investigator) for the duration of study treatment and 30 days after the
last dose of study treatment.

- Males should practice contraception for the duration of study treatment and 30 days
after the last dose of study treatment as follows: condom use and contraception by
female partner.

- Subject is in stable condition on the basis of medical history, physical examination,
and laboratory screening, as determined by the investigator.

- Subject is willing and able to comply with the protocol assessments and visits, in the
opinion of the study nurse/coordinator and the Investigator.

- Written informed consent is obtained prior to participating the study.

Exclusion Criteria:

- Expected to receive a lung transplant within 1 year from the start of the Treatment
Phase or on a lung transplant waiting list at the start of the Treatment Phase.

- Known explanation for interstitial lung disease

- Subjects on OFEV™/Nintedanib with a dose interruption due to significant adverse
events within 6 weeks of screening visits.

- Ongoing IPF treatments with investigational therapy

- Ongoing IPF treatments with Esbriet® (Pirfenidone)

- Immunosuppressants (i.e., Mycophenolate, Imuran, Cyclophosphamide), and cytokine
modulating agents within 1 month of Screening Visit and throughout the study

- Use of antibiotics and systemic steroids due to IPF exacerbation within 1 month of
Screening Visit

- Clinically significant cardiovascular disease, including myocardial infarct within
last 6 months, unstable ischemic heart disease, congestive heart failure or angina

- Resting pulse < 50 bpm, SA (sinoatrial) or AV (atrioventricular) block, uncontrolled
hypertension, or QTcF (QT interval corrected using the Fridericia formula) > 450 ms

- Immune system disease

- Any significant laboratory abnormality which, in the opinion of the Investigator, may
put the subject at risk

- History of malignancy < 5 years prior to signing the informed consent, except for
adequately treated basal cell or squamous cell skin cancer or in situ cervical cancer.

- History or evidence of drug or alcohol abuse

- History of HIV (human immunodeficiency virus) or other active infection.

- Currently has a clinically significant medical condition including the following:
neurological, psychiatric, immunological, metabolic, hepatic, hematological, pulmonary
(other than IPF) , cardiovascular (including uncontrolled hypertension),
gastrointestinal, urological disorder, or central nervous system (CNS) infection that
would pose a risk to the subject if they were to participate in the study or that
might confound the results of the study.

Note: Active medical conditions that are minor or well-controlled are not exclusionary if,
in the judgment of the Investigator, they do not affect risk to the subject or the study
results. In cases in which the impact of the condition upon risk to the subject or study
results is unclear, the Medical Monitor should be consulted.

- CYP2C8 (cytochrome P450 isoenzyme C28) and CYP2C9 (cytochrome P450 isoenzyme C29)
substrates with narrow therapeutic indices (i.e. paclitaxel, phenytoin and S-warfarin)
within 14 days of Screening Visit and throughout the study.

- Beta blockers within 14 days of Screening Visit and throughout the study

- Macrolide or quinolone class antibiotics within 14 days of Screening Visit and
throughout the study.

- Poor peripheral venous access that will limit the ability to draw blood as judged by
the Investigator.

- Currently participating, or has participated in, a study with an investigational or
marketed compound or device within 3 months prior to signing the informed consent.

- Unwilling or unable to conduct Spirometry (Vital Capacity) test.

- Unable to cooperate with any study procedures, unlikely to adhere to the study
procedures and keep appointments, in the opinion of the Investigator, or is planning
to relocate during the study.