AURA-LV: Aurinia Urinary Protein Reduction Active - Lupus With Voclosporin (AURA-LV)

Voclosporin is a next generation CNI intended for use in the prevention of organ graft
rejection and for the treatment of autoimmune diseases. The aim of the current study is to
investigate whether voclosporin added to the standard of care treatment in active LN is able
to reduce disease activity, as measured by a reduction in proteinuria. Two doses of
voclosporin will be studied and compared in a placebo controlled trial on a background of MMF
and corticosteroids. Patients with active, flaring LN will be eligible to enter the study.
They are required to have a diagnosis of LN according to established diagnostic criteria
(American College of Rheumatology) and clinical and biopsy features suggestive of active
nephritis. Efficacy will be assessed by the ability of the drug combination to reduce the
level of proteinuria while demonstrating an acceptable safety profile.

Inclusion Criteria:

Male or female subjects aged 18 to 75 years.

Diagnosis of systemic lupus erythematosus (SLE) according to the American College of
Rheumatology criteria.

Kidney biopsy within 6 months prior to Screening (Visit 1) with a histologic diagnosis of
lupus nephritis (International Society of Nephrology/Renal Pathology Society 2003
classification of lupus nephritis) Classes III, IV-S or IV-G, (A) or (A/C); or Class V,
alone or in combination with Class III or IV.

Laboratory evidence of active nephritis at screening, defined as:

- Class III, IV-S or IV-G: Confirmed proteinuria ≥1,500 mg/24 hours when assessed by 24
hour urine collection, defined by a UPCR of ≥1.5 mg/mg assessed in a first morning
void urine specimen (2 samples).

- Class V (alone or in combination with Class III or IV): Confirmed proteinuria ≥2,000
mg/24 hours when assessed by 24 hour urine collection, defined by a UPCR of ≥2 mg/mg
assessed in a first morning void urine specimen (2 samples).

Exclusion Criteria:

Estimated glomerular filtration rate (eGFR) as calculated by the Chronic Kidney Disease
Epidemiology Collaboration equation of ≤45 mL/min/1.73 m2.

Currently requiring renal dialysis (hemodialysis or peritoneal dialysis) or expected to
require dialysis during the study period.

A previous kidney transplant or planned transplant within study treatment period.

In the opinion of the Investigator, subject does not require long-term immunosuppressive
treatment (in addition to corticosteroids).

Current or medical history of:

- Pancreatitis or gastrointestinal hemorrhage within 6 months prior to screening.

- Active unhealed peptic ulcer within 3 months prior to screening. If an ulcer has
healed and the subject is on adequate therapy, the subject may be randomized.

- Congenital or acquired immunodeficiency.

- Clinically significant drug or alcohol abuse 2 years prior to screening.

- Malignancy within 5 years of screening, with the exception of basal and squamous cell
carcinomas treated by complete excision. Subjects with cervical dysplasia that is
cervical intraepithelial neoplasia 1, but have been treated with conization or loop
electrosurgical excision procedure, and have had a normal repeat PAP are allowed.

- Lymphoproliferative disease or previous total lymphoid irradiation.

- Severe viral infection (such as CMV, HBV, HCV) within 3 months of screening; or known
human immunodeficiency virus infection.

- Active tuberculosis (TB), or known history of TB/evidence of old TB if not taking
prophylaxis with isoniazid.

Other known clinically significant active medical conditions, such as:

- Severe cardiovascular disease including congestive heart failure, history of cardiac
dysrhythmia or congenital long QT syndrome.

- Liver dysfunction (aspartate aminotransferase, alanine aminotransferase, or bilirubin
greater than 2.5 times the upper limit of normal) at screening and confirmed before
randomization.

- Chronic obstructive pulmonary disease or asthma requiring oral steroids.

- Bone marrow insufficiency unrelated to active SLE (according to Investigator judgment)
with white blood cell count <2,500/mm3; absolute neutrophil count <1.3 x 103/μL;
thrombocytopenia (platelet count <50,000/mm3).

- Active bleeding disorders.

- Current infection requiring IV antibiotics.

Any overlapping autoimmune condition for which the condition or the treatment of the
condition may affect the study assessments or outcomes. Overlapping conditions for which
the condition or treatment is not expected to affect assessments or outcomes are not
excluded.

Subjects who are pregnant, breast feeding or, if of childbearing potential, not using
adequate contraceptive precautions.