• Type of GN commonly present in patients with systemic lupus erythematosus (SLE)
  • Characterized by deposition of circulating immune complexes in the glomerular tissue, leading to injury and inflammation
  • SLE patients with LN symptoms typically present with common signs of renal disease, such as proteinuria, hematuria, hypertension, and renal insufficiency
  • 30-50% of SLE patients will show clinical manifestations of renal disease during diagnosis
  • 80% of children and 60% of adults with SLE will develop renal aberrations at some point in their life
  • Gold standard for diagnosis is renal biopsy; however, the procedure is extremely invasive
    • Patient must first present with abnormal urinalysis and extrarenal manifestations of SLE before biopsy is obtained7
  • SLE etiology is unknown, but possible factors include genetic predisposition, environmental agents, and female hormones6
  • Incidence and prevalence for SLE and LN greatly vary based on geographic location, age, gender, and ethnicity
  • High female dominance for SLE
    • 10:1 female-to-male ratio (no gender preference for LN)
    • SLE usually diagnosed in women 15-45 years of age
  • LN is typically more severe in men and children
  • 3-4 times more common in people of African, Hispanic, or Asian ancestry for both SLE and LN
  • LN prognosis is typically good, though widely varies among patients; some will be asymptomatic, while others relapse multiple times
    • 8-15% of patients will suffer from ESRD6
  • MN gradually progresses and can be identified into a four-stage classification:
  • In 2004, the International Society of Nephrology and the Renal Pathology Society updated a classification system that describes the extent of disease in lupus nephritis
  • Classes I-II are associated with minimal renal manifestations and may not require treatment
  • Classes III-V are associated with aggressive, proliferative lesions
  • Class VI is associated with ESRD7

CNI: calcineurin inhibitor; ESRD: end-stage renal disease; MMF: mycophenolate mofetil.

Recommendations are based on the KDIGO Clinical
Practice Guideline for Glomerulonephritis

    • Treatment aimed towards remission of proteinuria to slow progression of ESRD
      • Prognosis goals: prevention of ESRD
    • Options are based on classifications of renal manifestations
      • Class I
        • No indication
      • Class II
        • Nephrotic levels of proteinuria – treatment with corticosteroids or CNIs
      • Class III/IV (initial)
        • Corticosteroids in combination with cyclophosphamide or MMF
      • Class III/IV (maintenance)
        • Corticosteroids in combination with AZA or MMF
        • If patient is intolerant, switch to corticosteroids in combination with CNIs
      • Class V
        • Non-nephrotic proteinuria levels – supportive therapy with renin-angiotensin system blockers
        • Nephrotic proteinuria levels – corticosteroids in combination with cyclophosphamide, CNIs, AZA or MMF
      • Class VI
        • No indication for therapy – dialysis or kidney transplant recommended7,10

      Alternative option not listed in 2012 KDIGO Guidelines

      • The product is an FDA approved treatment option11

    CNI: calcineurin inhibitor; ESRD: end-stage renal disease; MMF: mycophenolate mofetil.

Treatment Options
Immunosuppressive Therapy
  • Prednisone
  • Prednisolone
  • Methylprednisolone
  • Cytotoxic Agents10
  • Cyclophosphamide (Cytoxan®)
  • Azathioprine (Imuran®)
  • Calcineurin Inhibitors10
  • Cyclosporine (Gengraf®)
  • Tacrolimus (Prograf®)
  • Immunosuppressive Agents10
  • Mycophenolate mofetil (CellCept®)
  • Acthar® Gel)11*

    *FDA approved, but not enough contemporaneous data for KDIGO to make a use recommendation