Acthar for the Treatment of Systemic Lupus Erythematosus in Patients With a History of Persistently Active Disease

The first phase of the study is an 8-week randomized, double-blind, placebo-controlled,
parallel-group add-on pilot study exploring the efficacy, safety, and pharmacodynamics of
Acthar in SLE patients with a history of persistently active disease with arthritic and/or
cutaneous involvement despite standard of care, which includes chronic/stable prednisone use
for a minimum of 4 weeks prior to screening. Patients will be randomized to one of four
possible treatment groups (Acthar [low dose (40 U [0.5 mL]) or high dose (80 U [1.0 mL])] or
equivalent volumes of Placebo [low or high 'dose']) in a 2:1:2:1 ratio
(Acthar:Placebo:Acthar:Placebo). In Weeks 1-4, patients will administer study medication via
subcutaneous (SC) injection 0.5 mL daily or 1.0 mL every other day based on randomization.
In Weeks 5-8, patients will taper the study medication. Patients will continue on their
stable steroid regimen during this phase of the study.

After completion of Week 8 in the double-blind phase, patients may choose to participate in
the optional open-label phase where they will receive an Acthar maintenance regimen for 44
weeks. The initial Acthar regimen will be assigned based on the study medication regimen the
patient received at the completion of the double-blind phase (Visit 6, Week 8). Acthar
regimen may be adjusted based on the Investigator's judgment with the goal of achieving a
stable Acthar regimen no later than Week 28. Once the stable Acthar regimen is achieved, the
Investigator should consider tapering the steroid regimen to a low daily dose or completely
discontinue.

Inclusion Criteria:

- Male or female ≥ 18 years of age at screening who are able to provide informed
consent

- Diagnosis of SLE according to the American College of Rheumatology revised criteria
(fulfilled ≥ 4 criteria)

- Active SLE with arthritic and/or cutaneous involvement as demonstrated by a
SELENA-SLEDAI score ≥ 2 (clinical manifestation must include rash and/or arthritis)

- Moderate to severe rash and/or arthritis as demonstrated by BILAG score A or B in the
mucocutaneous and/or musculoskeletal body systems

- Documented history of autoantibodies to at least one of the following: anti-dsDNA,
anti-Smith, or anti-cardiolipin

- Documented history of positive antinuclear antibody (ANA)

- Currently on a stable dose of prednisone (7.5 to 30 mg/day of prednisone or
equivalent within the 4 weeks prior to screening). The prednisone regimen must remain
stable through the double-blind phase and until the stable Acthar regimen is attained
in the open-label phase.

Exclusion Criteria:

- Patients with a recent history (≤ 2 months prior to screening) of starting prednisone
(or equivalent) use

- Patients with active nephritis defined as serum creatinine > 2.5 mg/dL or protein
creatinine ratio (PCR) > 1.5 g/g, or patients that required hemodialysis within 3
months prior to screening

- Active central nervous system (CNS) lupus (including seizures, psychosis, organic
brain syndrome, cerebrovascular accident, cerebritis, or CNS vasculitis), requiring
therapeutic intervention within 3 months prior to screening

- Type 1 or type 2 diabetes mellitus (history of gestational diabetes mellitus is not
an exclusion), or patients currently taking hypoglycemic medication

- History of using certain medications prior to screening:

1. oral prednisone (or equivalent) > 30 mg/day, any steroid injection,
cyclosporine, or non-biologic investigational drug within 3 months prior to
screening

2. IVIg or plasmapheresis within 4 months prior to screening

3. cyclophosphamide within 6 months prior to screening; and/or

4. B-cell targeted therapy, abatacept, or any biologic investigational agent within
12 months prior to screening

- Contraindication per Acthar Prescribing Information: scleroderma, osteoporosis,
systemic fungal infections, ocular herpes simplex, recent surgery, history of or the
presence of peptic ulcer, congestive heart failure, uncontrolled hypertension,
primary adrenocortical insufficiency, or adrenal cortical hyperfunction

1. For the purposes of this study, osteoporosis is defined as evidence of vertebral
or long bone fracture or vertebral T-score > 2.0

2. For the purposes of this study, history of peptic ulcer is defined as ≤ 6 months
prior to screening

3. For the purposes of this study, congestive heart failure is defined as New York
Heart Association Functional Class III-IV