Inclusion Criteria:

- Male or female patients > 18 years of age, able to provide written informed consent.

- Diagnosis of Primary Sclerosing Cholangitis.

- Scheduled for an ERCP as part of their clinical care.

- Diagnosed with trisomy 7 on cytologic testing.

- Willingness to utilize adequate contraception (if female, evidenced by being
postmenopausal for at least 6 months or using contraceptive pill; for both females
and males, being surgically sterile, or using two forms of barrier contraception)
from screening to at least one month after the trial.

Exclusion Criteria:

- Cholangiocarcinoma, hepatocellular carcinoma, pancreatic adenocarcinoma or other
malignancy <=3 years of registration.

- Other liver disease as determined by standard clinical, serological, imaging or
histological criteria.

- Secondary cause of sclerosing cholangitis (IgG cholangiopathy, autoimmune,
post-surgical biliary stricture, radiation, human immunodeficiency syndrome).

- Cholestasis with a bilirubin of > 1.6 mg/dl (normal range: 0.1 - 1.0 mg/dL).

- Decompensated cirrhosis, Child-Pugh Class B or C.

- Child A cirrhosis with portal hypertension (i.e., splenomegaly, esophageal or gastric
varices, or platelet count < 100,000/µl [normal range: 150 - 450 x 103/µL]).

- Transaminase (AST [norm.: 8-48 U/L], ALT [norm.: 7-55 U/L]) elevation of more than
three times the upper limit of the normal range.

- Pregnancy.

- Nursing mothers.

- Uncontrolled intercurrent illness.

- Concurrent administration of CYP3A modulators, Antiepileptics, Rifampin, St. Johns
wort, Ketoconazole, protonpump-inhibitors.

- Men or women unwilling to employ adequate contraception.

- Abnormalities of the cornea by history.

- Moderate diarrhea defined as defecation frequency of equal or more than 4/d for those
with their colon, equal or more than 8/d for patients with a pouch, and high ostomy
output with those with ostomy.

- Known interstitial lung disease