• Characterized by mesangial deposits of IgG-IgA1 immune complexes, which have been found to induce a inflammatory response and lead to a loss of kidney function6
  • Patients typically present with either macroscopic hematuria and proteinuria or persistent asymptomatic microscopic hematuria7
    • Macroscopic hematuria typically follows a respiratory infection and presents with brown urine without clots
    • Microscopic hematuria patients are typically asymptomatic and must be diagnosed with a urine test. Proteinuria may or may not be present
  • Nephrotic syndrome is uncommon (only 5%)
  • Older patients diagnosed late in life may have suffer from acute kidney injury or chronic kidney disease
  • Incidence rate estimated to be about 2.5 patients/100,000 on average worldwide
  • Higher male dominance
    • 3:1 male-to-female ratio in Caucasians; 1:1 in Asians6
    • Most common in men in their 20s and 30s7
  • Most common in Asia and southern Europe, followed by northern Europe and North America7
  • Prognosis is typically good for most patients if proteinuria spilling is <0.2 g/24hr and normotensive
  • By age 20, ¼ of patients will suffer from ESRD
    • An additional 20% of those patients will have progressive decline in estimated glomerular filtration rate
    • Slow disease progression – highly dependent on hypertension and proteinuria levels6,7

    ESRD: end-stage renal disease.

Recommendations are based on the KDIGO Clinical
Practice Guideline for Glomerulonephritis

  • Treatment aimed towards remission of proteinuria to slow progression of ESRD
    • Prognosis goals: kidney survival and prevention of ESRD
  • Options are based on degree and persistence of proteinuria and kidney function
  • Initial therapy upon diagnosis is dependent on proteinuria levels
    • Non-nephrotic levels – supportive therapy of angiotensin-converting enzyme (ACE) inhibitors or angiotensin II receptor blockers (ARBs), followed by 6-month course of corticosteroids if proteinuria levels remain ≥1 g/day
    • Crescentic IgAN with rapidly deteriorating kidney function – corticosteroids combined with immunosuppressive therapy
  • Non-steroidal immunosuppressive agents have some benefits in reducing nephrotic levels of proteinuria, but lack evidence to be used as a first line of defense
    • Cyclophosphamide or azathioprine in combination with corticosteroids should only be used with rapidly deteriorating kidney function
  • As of 2012, the KDIGO guidelines list the following therapy as a potential treatment option, but there was insufficient data to make a recommendation for this agent:
    • Mycophenolate mofetil (MMF)10

Alternative option not listed in 2012 KDIGO Guidelines

  • The product is an FDA approved treatment option11

ESRD: end-stage renal disease.

Treatment Options
Immunosuppressive Therapy
  • Prednisone
  • Prednisolone
  • Cytotoxic Agents10
  • Cyclophosphamide (Cytoxan®)
  • Azathioprine (Imuran®)
  • Immunosuppressive Agents10
  • Mycophenolate mofetil (CellCept®)
  • Acthar® Gel11*

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