Liver Test Study of Using JKB-122 in AIH Patients

This is a Phase 2, pilot study in which JKB-122 is given once daily for 24 weeks to subjects
with AIH who have liver enzymes that are 1.25 to 10 times the upper limit of normal (ULN) and
who have had a failed response to, incomplete response to, intolerant to, ineligible to or
unwilling to take current immunosuppressant therapies. Current immunosuppressant therapy is
defined as prednisone or other steroids with or without azathioprine. Failed response is
defined as being non-responsive to or worsening in symptoms (elevated Alanine
Aminotransferase (ALT)/aspartate aminotransferase (AST) and/or total bilirubin) despite
compliance with the standard of care (SOC) over a 6 months period. Incomplete response is
defined as lack of a sustained normalization of elevated ALT/AST to current standard of care
(SOC) for at least 6 months. "Refractory" to the current therapy includes patients who had a
failed response to, or incomplete response to the current therapy.

Subjects will be at least 18 years of age, either male or female and will have been on
therapy for AIH and have not had a normalization of ALT with the current therapy.

Inclusion Criteria:

- Has definite or probable AIH diagnosis.

- Has had a liver biopsy or Fibroscan™ within 3 years and the severity of hepatic
dysfunction is limited to the following:

- Metavir Stage 0 to stage 3 fibrosis (according to liver biopsy) or Fibroscan™
results

- ALT and AST values not exceeding 10x ULN

- Normal bilirubin and prothrombin time (PT/INR)

- Has elevated liver test results (ALT) at least 1.25 x ULN and not exceeding 10 x ULN
at baseline.

- Has had a failed response, incomplete response, intolerant, ineligible or unwilling to
take current immunosuppressive therapies. Current immunosuppressive therapy is defined
as prednisone or other steroids with or without azathioprine.

Exclusion Criteria:

- Has history of allergy to JKB-122 or related compounds

- Has human immunodeficiency virus (HIV) or is hepatitis B virus or hepatitis C virus
(HCV) positive

- Has positive urine drug screen at Screening

- Has been diagnosed with other overlapping liver diseases such as primary biliary
cirrhosis (PBC) or primary sclerosing cholangitis (PSC)

- Is currently consuming alcoholic drinks greater than 25g/day and within 3 months prior
to the first screening visit.

- Is being treated with any prescription narcotic drug (including transdermal delivery
systems)

- Concurrent medications within 30 days prior to screening:

- Opioids

- Thioridazine

- Silymarin and related medications

- Potentially hepatotoxic drugs

- Has a known or suspected central nervous system disorder that may predispose to
seizures or lower the seizure threshold

- Has unstable and uncontrollable hypertension (>180/110 mmHg) or a blood pressure
reading at baseline of 150/90 mmHg on 2 occasions separated by a 30 minute interval

- Is currently receiving dietary supplements other than a multivitamin to treat AIH

- Has received other investigational agents within 90 days prior to the first screening
visit

- Has impaired renal function

- Has malignancy.

- If female, pregnant or lactating

- Has history of gastroesophageal varices, ascites, hepatic encephalopathy,
hepatocellular carcinoma, and s/p liver transplantation