• Characterized by fusion of podocyte foot processes and minimal glomerulus alterations, depicted as microscopic lipid droplets while observed under light microscopy8
  • Patients typically present with edema and nephrotic syndrome with accompanying acellular urinary casts7
    • Fluid retention often exceeds 3% of body weight6
  • Pathogenesis for idiopathic MCD is unknown – believed to be a consequence of cytokines that alter podocyte integrity and increase permeability to protein7
  • Secondary MCD may result from malignancies, drugs, or use of interferons
  • Causes nephrotic syndrome in about 90% of children <10 years of age, about 50-70% of older children, and 10-15% of adults
  • Incidence rate estimated to be about 2-7 children/100,000 on average worldwide
  • Higher male dominance
    • 2:1 male-to-female ratio
    • Most common in children ages 2-7
    • Less common in adults
  • Typically found in South Asians and Native Americans
  • Prognosis is usually good since MCD does not progress to renal failure6
    • However, relapses recur in 70-75% of children after first remission7

Recommendations based on the 2012 KDIGO
Clinical Practice Guideline for Glomerulonephritis

  • Treatment aimed towards remission of proteinuria
    • Prognosis goals: complete remission of nephrotic syndrome
  • Options are based on steroid-dependency and frequency of relapse
  • Initial therapy upon diagnosis should consist of daily or alternating-day doses of corticosteroids
    • 4-16 weeks of treatment until remission
  • Cyclophosphamide is first recommended for frequently relapsing patients that cannot tolerate high-dose corticosteroids or have contraindications
  • CNIs are prescribed if patients relapse despite cyclophosphamide or wish to preserve fertility
    • Options include cyclosporine and tacrolimus
  • MMF therapy is recommended for patients intolerant to corticosteroids, cyclophosphamide, and CNIs10

Alternative options not listed in 2012 KDIGO Guidelines

  • Chlorambucil provides similar efficacy to cyclophosphamide, but is not a preferable recommendation due to higher frequency of adverse effects
  • Rituximab studies have shown favorable results in significantly reducing relapse for patients unresponsive to previous treatment options6
  • The product is an FDA approved treatment option11

CNI: calcineurin inhibitor; MMF: mycophenolate mofetil.

Treatment Options
Immunosuppressive Therapy
  • Prednisone
  • Prednisolone
  • Cytotoxic Agents6,10
  • Chlorambucil (Leukeran®)
  • Cyclophosphamide (Cytoxan®)
  • Calcineurin Inhibitors10
  • Cyclosporine (Gengraf®)
  • Tacrolimus (Prograf®)
  • Immunosuppressive Agents10
  • Mycophenolate mofetil (CellCept®)
  • Monoclonal Antibodies6
  • Rituximab (Rituxan®)
  • Acthar® Gel11*

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