Open-Label Extension of RPC1063 as Therapy for Moderate to Severe Ulcerative Colitis

The trial is an open label extension study. Eligible patients from the RPC01-3101 and
RPC01-202 trials will have the option to enroll in this trial to receive study medication for
up to 5 years or until marketed approval

Inclusion Criteria:

1. Previously participated in a trial of RPC1063 for UC and meet the criteria for
participation in the open label extension as outlined in the prior trial

2. Female patients of childbearing potential:

Must agree to practice a highly effective method of contraception throughout the trial
until completion of the safety follow-up visit. Highly effective methods of
contraception are those that alone or in combination result in a failure rate of a
Pearl index of less than 1% per year when used consistently and correctly. Acceptable
methods of birth control in the trial are the following:

- combined hormonal (oestrogen and progestogen containing) contraception, which may
be oral, intravaginal, or transdermal

- progestogen-only hormonal contraception associated with inhibition of ovula-tion,
which may be oral, injectable, or implantable

- placement of an intrauterine device (IUD)

- placement of an intrauterine hormone-releasing system (IUS)

- bilateral tubal occlusion

- vasectomised partner

- sexual abstinence

Male patients:

Must agree to use a latex condom during sexual contact with women of childbearing
po-tential while participating in the trial until completion of the safety follow-up
visit.

All patients:

Periodic abstinence (calendar, symptothermal, post-ovulation methods), withdrawal
(coitus interruptus), spermicides only, and lactational amenorrhoea method are not
ac-ceptable methods of contraception. Female condom and male condom should not be used
together.

3. Must provide written informed consent and have the ability to be compliant with the
schedule of protocol assessments, which must be obtained prior to any trial-related
procedures.

Exclusion Criteria:

1. Have received any of the following therapies since the first dose of investigational
drug in the prior RPC1063 trial:

- Treatment with a biologic agent

- Treatment with an investigational agent other than RPC1063

- Treatment with D-penicillamine, leflunomide, thalidomide, natalizumab or
fingolimod

- Treatment with lymphocyte-depleting therapies (e.g., Campath, anti-CD4,
cladribine, rituximab, ocrelizumab, cyclophosphamide, mitoxantrone, total body
irradiation, bone marrow transplantation, alemtuzumab, daclizumab)

- Treatment with a live vaccine within 4 weeks prior to Visit 1 of this trial

2. Are currently receiving or require initiation of any of the following therapies:

- Treatment with corticosteroids at a dose that exceeds the prednisone equivalent
of >40 mg

- Treatment with immunosuppressive agents (eg, azathioprine, 6-MP, or methotrexate)

- Chronic non-steroidal anti-inflammatory drug (NSAID) use (Note: occasional use of
NSAIDs and acetaminophen [eg, headache, arthritis, myalgias, or menstru-al
cramps] and aspirin up to 325 mg/day is permitted)

- Treatment with Class Ia or Class III anti-arrhythmic drugs or treatment with two
or more agents in combination known to prolong PR interval

3. Pregnancy, lactation, or a positive serum beta human chorionic gonadotropin (hCG)

4. Clinically relevant hepatic, neurological, pulmonary, ophthalmological, endocrine,
psychiatric or other major systemic disease making implementation of the protocol or
interpretation of the trial difficult or that would put the patient at risk by
participating in the trial or that would have required a patient to discontinue
treatment in previous RPC1063 trial

5. Clinically relevant cardiovascular conditions, including history or presence of recent
myocardial infarction, unstable angina, stroke, transient ischemic attack,
decompensated heart failure requiring hospitalization, Class III/IV heart failure,
sick sinus syndrome, or severe untreated sleep apnea

6. Liver function impairment or persisting elevations of aspartate aminotransferase (AST)
or alanine aminotransferase (ALT) > 5 times the upper limit of normal (ULN), or direct
bilirubin > 3 times the ULN

7. FEV1 or FVC < 50% of predicted values