• 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 levels^6,7

  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
Corticosteroids10 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