Acthar SLE (Systemic Lupus Erythematosus)
Acthar SLE (Systemic Lupus Erythematosus)
This is a randomized study exploring the efficacy, safety and steroid sparing ability of two
 doses (40 U and 80 U) of Acthar in SLE patients with immune mediated hematologic
 manifestations requiring steroid use for a minimum of 2 weeks prior to screening.
Acthar is currently labeled for use during an exacerbation or for maintenance therapy in
 selected SLE cases, however data from prospective trials on hematologic manifestations of SLE
 are not available. Due to the potential effect of lowering or eliminating autoantibodies, the
 absence of bone marrow suppression, and a steroid sparing effect, Acthar may represent a
 novel therapeutic option in recalcitrant cases of hematologic SLE.
Inclusion Criteria:

 -  Able to provide informed consent.

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

 -  Hematologic BILAG scores due to hemolytic anemia or thrombocytopenia of BILAG A
 (including hemolytic anemia with hemoglobin < 8.0 g/dL or platelet count < 25 x 109 l)
 or BILAG B (including hemolytic anemia with hemoglobin 8.0 - 9.9 g/dDL or platelet
 count 25 - 49 x 109 l) based on screening laboratory assessment.

 -  Currently taking prednisone ≥ 7.5 mg or equivalent for hematologic SLE for at least 2
 weeks prior to screening.

 -  Use of antimalarials and NSAIDs (stable regimen within the 4 weeks prior to
 screening), as well as methotrexate, azathioprine, and mycophenolate mofetil (stable
 regimen within the 4 weeks prior to screening) are permitted but not required. If
 used, the regimen must remain stable through the study. An increase or addition of SLE
 medication at any time during the study is not permitted.

 -  Ability to comply with study procedures, which include SC injections of study
 medication, adhering to concomitant medication restrictions, and attending scheduled
 office visits.

 Exclusion Criteria:

 -  Patients with a recent history (< 2 week prior to screening) of starting prednisone or
 equivalent.

 -  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 is not an
 exclusion), or patients currently taking hypoglycemic medication.

 -  Patients with a history of concomitant medication use as follows:

 a. Receipt of the following within 1 month prior to screening: i. Any steroid
 injection (IM, intraarticular, or IV) b. Receipt of any of the following within 3
 months prior to screening: i. Cyclosporine ii. Any non-biologic investigational drug
 c. Receipt of the following within 4 months prior to screening: i. IVIg ii.
 Plasmapheresis d. Receipt of cyclophosphamide within 6 months prior to screening e.
 Receipt of the following within 12 months prior to screening: i. B cell targeted
 therapy (rituximab or other anti-CD20 agent, anti-CD22 [epratuzumab], anti-CD52
 [alentuzumab], or belimumab) ii. Abatacept iii. Any biologic investigational agent

 -  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 lumbar spine T-score > 2.0 standard deviations below
 the mean of the reference population.

 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.

 4. For the purposes of this study, uncontrolled hypertension is defined as a mean
 systolic blood pressure ≥ 140 mm Hg and/or diastolic blood pressure ≥ 90 mm Hg on
 ≥ 3 seated readings taken at least 5 minutes apart during the screening period.

 -  Reproductive status:

 1. Women who are pregnant,

 2. Women who are breastfeeding,

 3. Women of childbearing potential who are unwilling or unable to use an acceptable
 method of birth control to avoid pregnancy for the entire study period, as
 evaluated by the Investigator (women of non-childbearing potential are those that
 have a history of hysterectomy, bilateral oophorectomy, or are postmenopausal
 with no history of menstrual flow for ≥ 12 months prior to screening).

 -  Immune System: Known immunocompromised status (not related to SLE or therapies for
 SLE), including individuals who have undergone organ transplantation or who are known
 to be positive for the human immunodeficiency virus.

 -  Patients with acute or chronic hepatitis C, or active hepatitis B infection, positive
 interferon gamma release assay (IGRA) or signs or symptoms concerning for active
 tuberculosis (TB); or use of antibiotics (antibacterial, antiviral, antifungal, or
 antiparasitic agent) within 4 weeks of randomization for treatment of an active
 infection.

 -  Presence of any other clinically significant disease or disorder which, in the opinion
 of the Investigator (by its nature or by being inadequately controlled), might put the
 patient at risk due to participation in the study, or may influence the results of the
 study or the patient's ability to complete the study.

 -  Any clinically significant laboratory abnormality, based on the Investigator's
 judgment. The following laboratory exclusions apply for all patients:

 1. AST > 2.5 times the upper limit of normal (ULN)

 2. ALT > 2.5 times ULN

 3. Hemoglobin A1c > 6.5%